Consults in Obstetric Anesthesiology

Suzanne K. W. Mankowitz Editor

Consults in Obstetric Anesthesiology

123

Consults in Obstetric Anesthesiology

Suzanne K. W. Mankowitz Editor

Consults in Obstetric Anesthesiology

Editor Suzanne K. W. Mankowitz, M.D. Associate Professor Department of Anesthesiology Columbia University Medical Center New York, NY USA

ISBN 978-3-319-59679-2    ISBN 978-3-319-59680-8 (eBook) https://doi.org/10.1007/978-3-319-59680-8 Library of Congress Control Number: 2018951064 © Springer International Publishing AG, part of Springer Nature 2018 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland

This book is dedicated in memory of my parents: to my father who taught me the importance of careful analysis, perseverance, and scientific discovery and to my mother whose curiosity and love of ideas live on in me. With special gratitude to my wonderful children, Benjamin, Zachary, Tamar, and Yaakov, whose support and understanding have made this project possible.

Preface

The goal of this book is to share information on the management of high-risk parturients. This endeavor was inspired by my colleagues at other institutions who informed me that no antepartum consultation service had been established at their hospitals. I recognized an unmet need and felt uniquely qualified to fill it. I have had the wonderful opportunity to meet many high-risk parturients in our antenatal anesthesiology consultation clinic. Our clinic has proven an essential part in our high-risk parturient care program, and it is the hope of this book that sharing our experience will help other programs. Some topics are meant to guide management for patients whom we treat daily such as those with obesity. Other topics relate to patients with more obscure diseases that may complicate pregnancy. A few sections address acute diseases that may occur unexpectedly on the labor and delivery floor. While much of this information can now be readily accessed online, there is a need to consolidate valid, detailed information on these topics in a format easily accessible to practitioners. For ease of use, these topics have been arranged alphabetically. My hope is that this project will allow residents, fellows, attending anesthesiologists and obstetricians to obtain enough information to develop the best anesthetic plans for our patients, to facilitate informed consent interviews, and to do so quickly and efficiently. I would like to give special thanks to my section authors for their hard work and dedication to this project and am grateful to my residents and fellows who inspire me every day. I would also like to thank Mr. Kumar Athiappan, my Project Coordinator at Springer, for his patience and guidance on this journey. Most importantly, I would like to thank my children for relinquishing time with me so that I could devote myself to others. Finally, we are deeply saddened by the sudden and untimely loss of Dr. Jerry Green, whose contributions were very much appreciated. New York, NY, USA

Suzanne K. W. Mankowitz

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Contents

1 Abnormal Placentation�������������������������������������������������������������������������������������������������   1 Joshua D. Younger and Laurence E. Ring 2 Achondroplasia�������������������������������������������������������������������������������������������������������������  5 Trevor Whitwell and Antonio Gonzalez Fiol 3 Acromegaly �������������������������������������������������������������������������������������������������������������������  9 Catherine Traill and Stephen H. Halpern 4 Acute Fatty Liver �������������������������������������������������������������������������������������������������������  13 Aiyuan Li and Weike Tao 5 Adrenal Insufficiency�������������������������������������������������������������������������������������������������  17 Catherine Traill and Stephen H. Halpern 6 Amphetamines and Other Stimulant Use�����������������������������������������������������������������  21 Curtis L. Baysinger 7 Amyotrophic Lateral Sclerosis�����������������������������������������������������������������������������������  25 Christopher K. Der and Jie Zhou 8 Anticoagulation�����������������������������������������������������������������������������������������������������������  29 Jeanette R. Bauchat and Joseph B. Bavaro 9 Antiphospholipid Syndrome �������������������������������������������������������������������������������������  35 Jonathan Paek and Shobana Chandrasekhar 10 Antithrombin Deficiency �������������������������������������������������������������������������������������������  39 James P. R. Brown and Joanne Douglas 11 Aortic Pathology���������������������������������������������������������������������������������������������������������  41 Paul D. Weyker and Christopher Allen-John Webb 12 Aortopulmonary and Cavopulmonary Shunts���������������������������������������������������������  45 Jean Marie Carabuena 13 Arnold-Chiari Malformation�������������������������������������������������������������������������������������  49 Megan Maxwell 14 Arrhythmias: Introduction ���������������������������������������������������������������������������������������  55 Scott Mankowitz 15 Arthrogryposis �����������������������������������������������������������������������������������������������������������  59 Antonio Gonzalez Fiol 16 Asthma�������������������������������������������������������������������������������������������������������������������������  63 Suzanne K. W. Mankowitz 17 Atrial Flutter and Fibrillation�����������������������������������������������������������������������������������  71 Scott Mankowitz ix

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18 Atrial Septal Defect�����������������������������������������������������������������������������������������������������  75 Jean Marie Carabuena 19 Atrial Switch, Arterial Switch, and Rastelli�������������������������������������������������������������  77 Jean Marie Carabuena 20 Atrioventricular Septal Defect, AV Canal ���������������������������������������������������������������  81 Jean Marie Carabuena 21 Autonomic Hyperreflexia�������������������������������������������������������������������������������������������  83 Hyndhavi Chowdary and Lesley Gilbertson 22 Benzodiazepine Use�����������������������������������������������������������������������������������������������������  87 Curtis L. Baysinger 23 Bernard–Soulier Syndrome���������������������������������������������������������������������������������������  89 Sharon Orbach-Zinger, Atara Davis, and Alexander Ioscovich 24 Bone Marrow Failure�������������������������������������������������������������������������������������������������  93 Andrea Girnius and Saulius Girnius 25 Brugada Syndrome�����������������������������������������������������������������������������������������������������  97 Scott Mankowitz 26 Budd–Chiari Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  101 Stephanie I. Byerly 27 Caffeine Use. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  105 Curtis L. Baysinger 28 Cannabis Use. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  107 Curtis L. Baysinger 29 Cardiac Conduction Blocks. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  109 Scott Mankowitz 30 Cardiac Sinus Arrhythmias. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  113 Scott Mankowitz 31 Cardiac Valvular Disorders: Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  115 Naola Austin 32 Cardiovascular Risk Assessment. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  119 Jean Marie Carabuena 33 Cerebral Aneurysm. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  121 Scott Mankowitz and Suzanne K. W. Mankowitz 34 Cerebral Arteriovenous Malformations. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  125 Scott Mankowitz and Suzanne K. W. Mankowitz 35 Charcot-Marie-Tooth. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  129 Patricia Dalby and Erica Coffin 36 Cholestasis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  133 Shannon E. Klucsarits 37 Cirrhosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  135 Jerry W. Green† 38 Cocaine Use. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  141 Curtis L. Baysinger

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39 Congenital Adrenal Hyperplasia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  145 Catherine Traill and Stephen H. Halpern 40 Conn Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  147 Catherine Traill and Stephen H. Halpern 41 Cushing Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  149 Catherine Traill and Stephen H. Halpern 42 Cystic Fibrosis������������������������������������������������������������������������������������������������������������� 153 Suzanne K. W. Mankowitz 43 Cysticercosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  157 David Wang and Suzanne K. W. Mankowitz 44 Diabetes Insipidus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  161 Catherine Traill and Stephen H. Halpern 45 Diabetic Ketoacidosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  163 Catherine Traill and Stephen H. Halpern 46 Disc Herniation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  165 Antonio Gonzalez Fiol 47 Double Outlet Right Ventricle. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  169 Jean Marie Carabuena 48 Ebstein’s Anomaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  173 Jean Marie Carabuena 49 Echinococcosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  175 J. Ross, L. Coleman, J. Murphy, and M. Zakowski 50 Ehler-Danlos . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  179 Fatemah Mamdani and Suzanne K. W. Mankowitz 51 Eisenmenger’s Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  185 Kristin Brennan and David Matthews Hatch 52 Eosinophilic Granulomatosis with Polyangiitis. . . . . . . . . . . . . . . . . . . . . . . . . . .  189 M. Zakowski, J. Murphy, J. Ross, and L. Coleman 53 Essential Thrombocythemia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  191 Jaime Aaronson 54 Ethyl Alcohol Use. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  193 Curtis L. Baysinger 55 External Cephalic Version. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  197 Robert M. Gaiser 56 Ex Utero Intrapartum Therapy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  199 Robert M. Gaiser 57 Factor V and Combined Factor V and VIII Deficiency . . . . . . . . . . . . . . . . . . . .  201 James P. R. Brown and Joanne Douglas 58 Factor V Leiden and Prothrombin Mutation: Common Thrombophilias . . . . .  205 James P. R. Brown and Joanne Douglas 59 Factor VII Deficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  209 James P. R. Brown and Joanne Douglas

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60 Factor X Deficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  211 James P. R. Brown and Joanne Douglas 61 Factor XI Deficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  213 James P. R. Brown and Joanne Douglas 62 Factor XIII Deficiency . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  217 James P. R. Brown and Joanne Douglas 63 Familial Dysautonomia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  219 Alaeldin A. Darwich 64 Fibrinogen Deficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  223 James P. R. Brown and Joanne Douglas 65 Glomerulonephritis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  227 Shannon E. Klucsarits 66 Glucose 6 Phosphate Deficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  231 Jerri Chen and Suzanne K. W. Mankowitz 67 Glycogen Storage Diseases. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  235 Janis M. Ferns and Stephen H. Halpern 68 Goodpasture Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  241 L. Coleman, J. Murphy, J. Ross, and M. Zakowski 69 Granulomatosis with Polyangiitis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  243 J. Murphy, J. Ross, L. Coleman, and M. Zakowski 70 Guillain-Barré Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  245 Christopher K. Der and Jie Zhou 71 Hallucinogen Use. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  249 Curtis L. Baysinger 72 Hematologic Malignancies. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  251 Andrea Girnius, Saulius Girnius, and Lesley Gilbertson 73 Hemolytic-Uremic Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  255 Michelle McCown Eddins 74 Hemophilia A and B. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  257 James P. R. Brown and Joanne Douglas 75 Henoch–Schonlein Purpura. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  261 Fatemah Mamdani and Suzanne K. W. Mankowitz 76 Hepatitis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  265 Jerry W. Green† 77 Hereditary Hemorrhagic Telangiectasia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  269 Patricia Dalby and Thomas J. Vernon 78 Hereditary Motor Sensory Neuropathies. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  273 Zaneta Strouch and Ashraf S. Habib 79 Homocystinuria. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  275 Janis M. Ferns and Stephen H. Halpern 80 Human Immunodeficiency Virus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  277 Roulhac D. Toledano

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81 Hyperosmolar Hyperglycemic State. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  283 Catherine Traill and Stephen H. Halpern 82 Hyperparathyroidism. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  285 Catherine Traill and Stephen H. Halpern 83 Hypertensive Diseases. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  289 Victoria Danhakl and Ruth Landau 84 Hypertrophic Cardiomyopathy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  295 Keren K. Griffiths and Suzanne K. W. Mankowitz 85 Hypoparathyroidism. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  299 Catherine Traill and Stephen H. Halpern 86 Illicit Drug Use and Substance Abuse. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  303 Curtis L. Baysinger 87 Immunoglobulin A Deficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  307 Kyra Bernstein and Suzanne K. W. Mankowitz 88 Inherited Bleeding Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  309 James P. R. Brown and Joanne Douglas 89 Inherited Thrombophilias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  315 James P. R. Brown and Joanne Douglas 90 Insulin Pump. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  319 Catherine Traill and Stephen H. Halpern 91 Intracranial Hypertension. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  323 Rakesh B. Vadhera and Rovnat Babazade 92 Intracranial Mass Lesions. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  329 Mohammed Faysal Malik, Ankit Patel, and Ami Attali 93 Jehovah’s Witnesses. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  335 Shobana Chandrasekhar 94 Kawasaki Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  339 Paul D. Weyker and Christopher Allen-John Webb 95 Klippel-Trenaunay Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  343 Adam M. Gerber and Suzanne K. W. Mankowitz 96 Left Ventricular Assist Devices (LVAD). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  347 Ami Attali, Ashley Taylor, and Michael Isley 97 Long QT Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  351 Scott Mankowitz 98 Lysosomal Storage Diseases. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  357 Janis M. Ferns and Stephen H. Halpern 99 Malignancy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  361 Roulhac D. Toledano 100 Malignant Hyperthermia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  365 Julia R. Menshenina and Suzanne K. W. Mankowitz 101 Marfan Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  369 Yang Long and Suzanne K. W. Mankowitz

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102 Mast Cell Activation Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  375 Andrea Girnius and Saulius Girnius 103 Moyamoya Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  379 Ami Attali, Asma Asif, and Stephanie Cook 104 Multiple Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  383 Christopher K. Der and Jie Zhou 105 Myasthenia Gravis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  387 Christopher K. Der and Jie Zhou 106 MYH9-Related Platelet Disorders. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  391 Sharon Orbach-Zinger, Atara Davis, and Alexander Ioscovich 107 Myopathies: Congenital, Metabolic and Mitochondrial, and Channelopathies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  395 Chantal Crochetiere and Elsa Lidzborski 108 Myopathies: Muscular Dystrophy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  399 Chantal Crochetiere and Elsa Lidzborski 109 Myxedema. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  405 Catherine Traill and Stephen H. Halpern 110 Nephrotic Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  409 Stephanie I. Byerly 111 Neurofibromatosis (NF) in Pregnancy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  413 Asma Asif, Stephanie Cook, and Ami Attali 112 Obesity in Pregnancy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  419 Laurence E. Ring 113 Obstructive Sleep Apnea (OSA) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  423 Suzanne K. W. Mankowitz and Anthony Fernandes 114 Opioids. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  429 Curtis L. Baysinger 115 Osteogenesis Imperfecta. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  433 Antonio Gonzalez Fiol 116 Pacemakers and Implanted Cardiac Defibrillators. . . . . . . . . . . . . . . . . . . . . . . .  437 Scott Mankowitz 117 Panhypopituitarism . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  441 Catherine Traill and Stephen H. Halpern 118 Parkinson’s Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  445 Jie Luo and Jie Zhou 119 Paroxysmal Supraventricular Tachycardia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  449 Scott Mankowitz 120 Patent Ductus Arteriosus (PDA). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  453 Jean Marie Carabuena 121 Peripartum Cardiomyopathy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  455 Mahesh Vaidyanathan and Paloma Toledo 122 Phenylketonuria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  459 Janis M. Ferns and Stephen H. Halpern

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123 Pheochromocytoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  461 Emily E. Sharpe 124 Pituitary Adenoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  465 Catherine Traill and Stephen H. Halpern 125 Polycythemia Vera . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  469 Andrea Girnius and Lesley Gilbertson 126 Porphyria. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  471 Janis M. Ferns and Stephen H. Halpern 127 Postpoliomyelitis Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  475 Jie Luo and Jie Zhou 128 Premature Cardiac Contractions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  477 Scott Mankowitz 129 Prior Anesthetic Problems. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  479 Susan Cosgrove and Richard Smiley 130 Prolactinomas . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  485 Catherine Traill and Stephen H. Halpern 131 Protein C and S Deficiencies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  489 James P. R. Brown and Joanne Douglas 132 Prothrombin (Factor II) Deficiency. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  493 James P. R. Brown and Joanne Douglas 133 Pulmonary Fibrosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  495 J. Nguyen, L. Coleman, and M. Zakowski 134 Pulmonary Hypertension in Pregnancy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  499 Kristin Brennan and David Matthews Hatch 135 Renal Disease and the Parturient on Dialysis. . . . . . . . . . . . . . . . . . . . . . . . . . . . .  503 Shannon E. Klucsarits 136 Restrictive Lung Disease and Pneumothoraces. . . . . . . . . . . . . . . . . . . . . . . . . . .  507 Katherine Gelber and Stephanie Goodman 137 Rheumatoid Arthritis in Pregnancy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  511 Suman Rajagopalan 138 Sarcoidosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  515 Suzanne K. W. Mankowitz 139 Scoliosis: Repaired and Unrepaired . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  517 Robert Jee and Edward T. Crosby 140 Seizure Disorder. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  523 Jon Dean Samuels 141 Sickle Cell Disease and Anesthesia Management in Laboring Women. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  529 Limor Sharoni Golzarpoor and Carolyn F. Weiniger 142 Single Ventricle. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  535 Jean Marie Carabuena 143 Solvents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  537 Curtis L. Baysinger

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144 Spina Bifida. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  539 Antonio Gonzalez Fiol 145 Spinal Cord Injury. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  543 Jie Luo and Jie Zhou 146 Spinal Cord Stimulators. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  547 Vanny Le 147 Spinal Muscular Atrophy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  551 Adam Sachs 148 Spondylolysis and Spondylolisthesis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  555 Antonio Gonzalez Fiol 149 Sticky Platelet Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  557 Sharon Abramovitz 150 Stroke During Pregnancy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  559 Elena Reitman 151 Sturge-Weber. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  565 Fatemah Mamdani and Suzanne K. W. Mankowitz 152 Syndrome of Inappropriate Antidiuretic Hormone. . . . . . . . . . . . . . . . . . . . . . . .  569 Catherine Traill and Stephen H. Halpern 153 Syringomyelia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  571 Adam Sachs and Azuka Onye 154 Systemic Lupus Erythematosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  575 Suman Rajagopalan 155 Systemic Sclerosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  579 Jonathan Paek and Shobana Chandrasekhar 156 Tethered Cord Syndrome. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  583 Minxian Liang and Jie Zhou 157 Tetralogy of Fallot (TOF). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  587 Jean Marie Carabuena 158 Thalassemia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  589 Xiwen Zheng and Suzanne K. W. Mankowitz 159 Thrombocytopenia: An Introduction. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  593 Joshua Zevy Hamburger and Yaakov Beilin 160 Thrombocytopenia: Gestational, Idiopathic, and Preeclampsia . . . . . . . . . . . . .  597 Joshua Zevy Hamburger and Yaakov Beilin 161 Thromboembolic Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  601 Andrea Girnius and Lesley Gilbertson 162 Thrombotic Thrombocytopenic Purpura. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  605 Joshua Zevy Hamburger and Yaakov Beilin 163 Thyroid Storm. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  609 Catherine Traill and Stephen H. Halpern 164 Tobacco . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  613 Suzanne K. W. Mankowitz

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165 Transplantation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  615 Kerri M. Wahl and Holly A. Muir 166 Transposition of the Great Arteries (TGA) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  621 Jean Marie Carabuena 167 Transverse Myelitis����������������������������������������������������������������������������������������������������� 625 Elvedin Lukovic and Suzanne K. W. Mankowitz 168 Transversus Abdominis Plane Block. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  629 Ramon Go, Laura Lombardi, and Christopher Allen-John Webb 169 Truncus Arteriosus. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  633 Jean Marie Carabuena 170 Tuberous Sclerosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  635 Minxian Liang and Jie Zhou 171 Twin-Twin Transfusion Syndrome and Laser Ablation . . . . . . . . . . . . . . . . . . . .  639 Robert M. Gaiser 172 Valvular Heart Disease: Regurgitant Lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . .  641 Naola Austin 173 Valvular Heart Disease: Stenotic Lesions. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  643 Naola Austin 174 Vascular Malformations of the Spinal Cord. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  647 Jon Dean Samuels 175 Ventricular Septal Defect. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  651 Jean Marie Carabuena 176 Ventricular Tachycardia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  653 Scott Mankowitz 177 Ventriculoperitoneal Shunts. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  655 Scott Mankowitz 178 Von Hippel–Lindau Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  659 Jie Zhou and Xiaoran Li 179 von Willebrand Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  663 James P. R. Brown and Joanne Douglas 180 Wilson’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  667 Stephanie I. Byerly 181 Wolff-Parkinson-White Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  671 Scott Mankowitz Index. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  675

Contributors

Jaime Aaronson, M.D.  Department of Anesthesiology, New York Presbyterian Weill Cornell Medicine, New York, NY, USA Sharon Abramovitz, M.D. Department of Anesthesiology, Weill Cornell Medicine, New York, NY, USA Asma Asif, D.O., M.B.A., M.D. Department of Anesthesiology, Wayne State University (WSU) School of Medicine, Henry Ford Hospital, Detroit, MI, USA Ami Attali, M.D., D.O. Department of Anesthesiology, Wayne State University (WSU) School of Medicine, Henry Ford Hospital, Detroit, MI, USA Naola Austin, M.D. Stanford Department of Anesthesiology, Perioperative and Pain Medicine, Stanford University Medical Center, Stanford Healthcare and Lucile Packard Children’s Hospital, Stanford, CA, USA Rovnat Babazade, M.D. Department of Anesthesiology, University of Texas Medical Branch, Galveston, TX, USA Jeanette R. Bauchat, M.D., M.S.  Division of Obstetric Anesthesiology, Vanderbilt University Medical Center, Nashville, TN, USA Joseph B. Bavaro, M.D., M.S. Department of Anesthesiology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA Curtis L. Baysinger, M.D.  Department of Anesthesiology, Vanderbilt University Medical Center, Vanderbilt University Hospital, Nashville, TN, USA Yaakov Beilin, M.D.  Department of Anesthesiology, Perioperative and Pain Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, USA Department of Obstetrics, Gynecology, and Reproductive Science, Icahn School of Medicine at Mount Sinai, New York, NY, USA Kyra Bernstein, B.A., M.D.  Department of Anesthesiology, New York Presbyterian, New York, NY, USA Kristin Brennan, M.D.  Department of Anesthesiology and Perioperative Medicine, PennState Health Milton S. Hershey Medical Center, Hershey, PA, USA James P. R. Brown, MBChB, MRCP, FRCA, MD, FRCPC UBC Department of Anesthesiology, Pharmacology and Therapeutics, BC Women’s Hospital, Vancouver, BC, Canada Department of Anesthesia, BC Women’s Hospital, Vancouver, BC, Canada Stephanie I. Byerly, M.D.  Department of Anesthesiology and Pain Management, University of Texas Southwestern, Dallas, TX, USA

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Jean Marie Carabuena, M.D. Department of Anesthesiology, Perioperative and Pain Medicine, Harvard Medical School, Brigham and Women’s Hospital, Boston, MA, USA Shobana Chandrasekhar, M.D. Department of Anesthesiology, Baylor Medical Center, Houston, TX, USA Jerri Chen, M.D.  Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Hyndhavi Chowdary, M.D.  UC Department of Anesthesiology, University of Cincinnati, Cincinnati, OH, USA Erica Coffin, M.D.  Department of Anesthesiology, Magee-Womens Hospital of University of Pittsburgh Medical Center, Pittsburgh, PA, USA L. Coleman, M.D.  Department of Anesthesiology, Charles Drew University of Medicine and Science, Los Angeles, CA, USA Department of Anesthesiology, Cedars-Sinai Medical Center, Los Angeles, CA, USA Stephanie Cook, M.D., D.O.  Department of Anesthesiology, Henry Ford Hospital, Detroit, MI, USA Susan Cosgrove, M.D. Department of Anesthesiology, Washington University, St. Louis, MO, USA Chantal Crochetiere, M.D.  Department of Anesthesia, Sainte-Justine Hospital, Montréal, QC, Canada Edward T. Crosby, M.D.  Faculty of Medicine, Department of Anesthesiology, University of Ottawa, Ottawa, ON, Canada Patricia Dalby, M.D.  Department of Anesthesiology, Magee-Womens Hospital of University of Pittsburgh Medical Center, Pittsburgh, PA, USA Victoria Danhakl, M.D. Department of Anesthesiology, Center for Precision Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA Alaeldin A. Darwich, M.D. Department of Anesthesiology, Weill Cornell Medicine, New York Presbyterian Hospital, New York, NY, USA Atara Davis, B.A., M.D. Department of Anesthesia, Rabin Medical Center, Beilinson Hospital, Petach Tikvah, Israel Christopher K. Der, M.D.  Department of Anesthesiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA Joanne Douglas, CM, MD, FRCPC  UBC Department of Anesthesiology, Pharmacology and Therapeutics, BC Women’s Hospital, Vancouver, BC, Canada Michelle McCown Eddins, M.D. Department of Anesthesiology and Pain Management, University of Texas Southwestern Medical Center, Dallas, TX, USA Anthony Fernandes, M.D. Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Janis M. Ferns, M.B.B.Chir., F.R.C.A. Department of Anesthesia, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON, Canada Antonio Gonzalez Fiol, M.D. Department of Anesthesiology, Yale University School of Medicine, New Haven, CT, USA Robert M. Gaiser, M.D., M.S.Ed.  Department of Anesthesiology, University of Kentucky Medical Center, Lexington, KY, USA

Contributors

Contributors

xxi

Katherine Gelber, M.D.  Department of Anesthesiology, Cedars Sinai Medical Center, Los Angeles, CA, USA Adam M. Gerber, M.D., Ph.D.  Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Lesley Gilbertson, M.D.  Division Director Obstetric Anesthesia, University of Cincinnati Medical Center and West Chester Hospital, Cincinnati, OH, USA Andrea Girnius, M.D. Department of Anesthesiology, University of Cincinnati Medical Center, Cincinnati, OH, USA Saulius Girnius, M.D. Division of Hematology/Oncology, Department of Medicine, University of Cincinnati Medical Center, Cincinnati, OH, USA Stephanie Goodman, M.D.  Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Ramon Go, M.D.  Division of Regional Anesthesia and Acute Pain Management, Department of Anesthesiology and Critical Care, Columbia University Medical Center, New York, NY, USA Jerry W. Green, D.O.  Department of Anesthesiology and Pain Management, University of Texas Southwestern Medical Center, Parkland Hospital, Dallas, TX, USA Keren K. Griffiths, M.D., Ph.D.  Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Ashraf S. Habib, M.B.B.Ch., M.Sc., M.H.Sc., M.D. Division of Women’s Anesthesia, Department of Anesthesiology, Duke University Medical Center, Durham, NC, USA Stephen H. Halpern, M.D.  Department of Anesthesia, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON, Canada Joshua Zevy Hamburger, M.D.  Department of Anesthesiology, Perioperative and Pain Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, USA David Matthews Hatch, M.D., M.B.A.  Department of Anesthesiology, Wake Forest Baptist Hospital, Winston Salem, NC, USA Alexander Ioscovich, M.D. Department of Anesthesia, Shaare Zedek Medical Center, Hebrew University, Jerusalem, Israel Michael Isley, M.D.  Division of Obstetrical Anesthesiology, Wayne State University (WSU) School of Medicine, Henry Ford Hospital, Detroit, MI, USA Robert Jee, M.D.  Faculty of Medicine, Department of Anesthesiology, University of Ottawa, Ottawa, ON, Canada Shannon E. Klucsarits, M.D. Department of Anesthesiology and Pain Management, University of Texas Southwestern, Parkland Hospital, Dallas, TX, USA Ruth Landau, M.D.  Department of Anesthesiology, Center for Precision Medicine, Columbia University College of Physicians and Surgeons, New York, NY, USA Vanny Le, M.D. Department of Anesthesiology, Rutgers-New Jersey Medical School, Newark, NJ, USA Aiyuan Li, M.D.  Department of Anesthesiology and Critical Care, Hunan Provincial Women and Children’s Hospital, Changsha, Hunan, China Minxian Liang, M.D. Department of Anesthesiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

xxii

Elsa Lidzborski, M.D.  Department of Anesthesia, Pitié-Salpêtrière Hospital, Paris, France Xiaoran Li, M.D., M.S.  Department of Anesthesiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA Laura Lombardi, M.D. Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Yang Long, M.D.  Department of Anesthesiology, NewYork-Presbyterian Hospital, Columbia University Medical Center, New York, NY, USA Elvedin Lukovic, M.D., Ph.D.  Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Jie Luo, M.D. Department of Anesthesiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA Mohammed Faysal Malik, M.D.  Department of Anesthesiology, Wayne State University (WSU) School of Medicine, Henry Ford Hospital, Detroit, MI, USA Fatemah Mamdani, M.D., M.P.H. Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Scott Mankowitz, M.D.  Emergency Medical Associates, Parsippany, NJ, USA Emergency Medicine Specialist, East Orange General Hospital, East Orange, NJ, USA Suzanne K. W. Mankowitz, M.D. Associate Professor, Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Megan Maxwell, M.D.  Department of Anesthesiology, University of Texas Southwestern Medical Center, Clements University Hospital, Dallas, TX, USA Julia R. Menshenina, M.D.  California Pacific Medical Center, San Francisco, CA, USA Holly A. Muir, M.D. Department of Anesthesiology, Duke University Medical Center, Durham, NC, USA J. Murphy, M.D.  Department of Anesthesiology, Charles Drew University of Medicine and Science, Los Angeles, CA, USA Department of Anesthesiology, Cedars-Sinai Medical Center, Los Angeles, CA, USA J. Nguyen, M.D.  Department of Anesthesiology, Cedars-Sinai Medical Center, Los Angeles, CA, USA Azuka Onye, D.O., M.D.  Department of Anesthesiology, University of Connecticut Medical Center, Farmington, CT, USA Sharon Orbach-Zinger, M.D.  Department of Anesthesia, Rabin Medical Center, Beilinson Hospital, Petach Tikvah, Israel Jonathan Paek, M.D.  Department of Anesthesiology, Baylor Medical Center, Houston, TX, USA Baylor University School of Medicine, Houston, TX, USA Ankit Patel, M.D.  Department of Anesthesiology, Wayne State University (WSU) School of Medicine, Henry Ford Hospital, Detroit, MI, USA Suman Rajagopalan, M.D. Department of Anesthesiology, Baylor College of Medicine, Houston, TX, USA Elena Reitman, M.D.  Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA

Contributors

Contributors

xxiii

Laurence E. Ring, M.D.  Division of Obstetric Anesthesiology, Department of Anesthesiology, Columbia University, New York, NY, USA J. Ross, M.D. Department of Anesthesiology, Charles Drew University of Medicine and Science, Los Angeles, CA, USA Department of Anesthesiology, Cedars-Sinai Medical Center, Los Angeles, CA, USA Adam Sachs, M.D. Department of Anesthesiology, University of Connecticut Medical Centre, Farmington, CT, USA Jon Dean Samuels, M.D.  Department of Anesthesiology, Weill Cornell Medicine, New YorkPresbyterian Hospital, New York, NY, USA Limor Sharoni Golzarpoor, M.D.  Department of Anesthesiology and Critical Care Medicine, Hadassah Hebrew University Medical Center, Jerusalem, Israel Department of Anesthesiology, Schneider Children’s Medical Center of Israel, Petah Tikva, Israel Emily E. Sharpe, M.D.  Department of Anesthesiology and Perioperative Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA Richard Smiley, M.D. Department of Anesthesiology, Columbia University College of Physicians and Surgeons, New York, NY, USA Zaneta Strouch, M.D. Division of Women’s Anesthesia, Department of Anesthesiology, Duke University Medical Center, Durham, NC, USA Weike Tao, M.D.  Department of Anesthesiology and Pain Management, The University of Texas Southwestern Medical Center, Dallas, TX, USA Ashley Taylor, M.D.  Wayne State University (WSU) School of Medicine, Henry Ford Hospital, Detroit, MI, USA Roulhac D. Toledano, M.D., Ph.D.  Department of Anesthesiology, Preoperative Care, and Pain Medicine, NYU Langone Hospital – Brooklyn, Brooklyn, NY, USA Paloma Toledo, M.D.  Department of Anesthesiology, Northwestern University, Chicago, IL, USA Catherine Traill, M.D. Department of Anesthesia, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON, Canada Rakesh B. Vadhera, M.D., F.R.C.A. Department of Anesthesiology, University of Texas Medical Branch, Galveston, TX, USA Mahesh Vaidyanathan, M.D. Department of Anesthesiology, Northwestern University, Chicago, IL, USA Thomas J. Vernon, M.D. Department of Anesthesiology, Magee-Womens Hospital of University of Pittsburgh Medical Center, Pittsburgh, PA, USA Kerri M. Wahl, M.D. Department of Anesthesiology, Duke University Medical Center, Durham, NC, USA David Wang, M.D. Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Christopher Allen-John Webb, M.D. Regional Anesthesia and Acute Pain Medicine, Department of Anesthesiology, Kaiser Permanente South San Francisco, South San Francisco, CA, USA Carolyn F. Weiniger, M.D. Department of Anesthesiology and Critical Care Medicine, Hadassah Hebrew University Medical Center, Jerusalem, Israel Division of Anesthesia and Critical Care and Pain, Tel Aviv Medical Center, Tel Aviv, Israel

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Paul D. Weyker, M.D. Department of Anesthesiology, Kaiser Permanente South San Francisco, South San Francisco, CA, USA Trevor Whitwell, M.D.  Rutgers-New Jersey Medical School, Newark, NJ, USA Joshua D. Younger, M.D.  Department of Anesthesiology, Pain Medicine, and Perioperative Medicine, Henry Ford Health System, Wayne State University School of Medicine, Detroit, MI, USA M. Zakowski, M.D.  Department of Anesthesiology, Charles Drew University of Medicine and Science, Los Angeles, CA, USA Department of Obstetric Anesthesiology, Charles Drew University of Medicine and Science, Los Angeles, CA, USA Xiwen Zheng, M.D.  Department of Anesthesiology, Columbia University Medical Center, New York, NY, USA Jie Zhou, M.D., M.S., M.B.A. Department of Anesthesiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

Contributors

1

Abnormal Placentation Joshua D. Younger and Laurence E. Ring

Background and Epidemiology Definition Placenta accreta has been used broadly to describe abnormal placentation with placental invasion into or beyond the myometrium of the uterus. When the placenta implants into the myometrium alone, it is termed placenta increta, and when it invades beyond the myometrium into the uterine serosa or even into the adjacent abdominal organs, it is termed placenta percreta [1].

Incidence The incidence of placenta accreta was recorded as 1/4000 in the 1970s and as high as 1/500 in the early 2000s [2]. This steady and steep increase has mirrored the rising cesarean delivery (CD) rate, which has risen from 5% in the 1970s to 30% in the modern day [3].

Risk Factors 1. CD has the greatest association with the risk of accreta; however, others include myomectomies, dilation and curettages, and ablation procedures. Prior uterine surgery or any pathology that has disrupted the uterus’s normal architecture also increases risk [1].

J. D. Younger, M.D. (*) Department of Anesthesiology, Pain Medicine, and Perioperative Medicine, Henry Ford Health System, Wayne State University School of Medicine, Detroit, MI, USA e-mail: [email protected] L. E. Ring, M.D. Division of Obstetric Anesthesiology, Department of Anesthesiology, Columbia University, New York, NY, USA e-mail: [email protected]

2. Placenta previa, an independent risk factor for placenta accreta, is a placenta that occludes the internal cervical os. The risk of accreta is anywhere between 1 and 5% in this population. Patients with a history of prior uterine surgery with a placenta previa are at increased risk of accreta. One study concluded that placenta previa increases the risk of an accreta, correlating with the number of prior CDs. This ranged from 3%, for the first repeat CD, to 67% in a patient undergoing her fifth CD [4] (see Table 1.1). 3. A history of four or fewer CDs, in the absence of previa, imbues a 14 μmol/L 340 μmol/L (5.7 mg/dL) >11 × 109/L Ascites or “bright liver” >42 U/L >47 μmol/L >150 μmol/L PT > 14s or APTT > 34s Microvesicular steatosis

3. The Swansea criteria are commonly used to establish the diagnosis [1]. See Table 4.1. AFLP is diagnosed if six or more categories are positive. 4. Liver biopsy is no longer routinely used due to the risk of severe bleeding, and the value of imaging studies remains to be determined.

Management 1. Early diagnosis and delivery are key to a good outcome. (a) It is recommended that delivery should be completed within 7 days of suspected diagnosis. Maternal survival approaches 100% if delivery is completed within 7 days of diagnosis. (b) Maternal survival decreases to 70% if delivery is completed later than 2 weeks after diagnosis. (c) In severe cases, urgent cesarean delivery is usually performed. Cesarean delivery may result in improved maternal and fetal survival rates. (d) Either regional or general anesthesia can be administered. The extent of coagulopathy and encephalopathy will dictate anesthetic management. One study reported the use of anesthesia in 28 cesarean deliveries: 13 combined spinal-epidurals (CSE), 3 spinals, and 12 general anesthetics were performed with no anesthetic complications reported. Of note, two of the patients who had CSE had an INR > 2.1 though this practice cannot be endorsed. 2. Treatment for AFLP is generally supportive. (a) Treatment involves controlling hypertension, providing caloric supplementation, supporting renal function, and correcting hypoglycemia, electrolyte and acid-base disturbances, and hypoproteinemia. Admission to an intensive care unit is advised.

4  Acute Fatty Liver

(b) Coagulopathy may require vitamin K, fresh frozen plasma, and cryoprecipitate transfusion. (c) Dialysis may be indicated in severe renal dysfunction and elevated ammonia levels. (d) Maternal lipid metabolism and mental status must be followed. If applicable, diet should be low in lipids and high in carbohydrates. FAO inhibitors, such as nonsteroidal anti-inflammatory drugs and valproic acid, should be avoided [8]. (e) Prophylactic antibiotics are often administered. (f) Liver transplant may be required. AFLP may deteriorate postpartum but typically gradually improves. However, some patients with refractory liver failure may require transplant. Patients with encephalopathy and increased lactate and ammonia levels on admission may be more likely to require transplant. 3. With early recognition and delivery, the prognosis has significantly improved over the past few decades. There is temporary worsening of hepatic and renal function within 48 h of delivery, but improvement should follow thereafter [2, 9]. 4. Fetuses born to mothers with AFLP should be maintained on a high-carbohydrate, low-fat diet and remain unfasted for more than 4 h. Aspirin, nonsteroids, tetracycline, and valproic acid should be avoided until neonatal status is established.

15

References 1. Knight M, Nelson-Piercy C, Kurinczuk JJ, Spark P, Brocklehurst P, UK Obstetric Surveillance System. A prospective national study of acute fatty liver of pregnancy in the UK. Gut. 2008;57:951–6. 2. Usta IM, Barton JR, Amon EA, Gonzalez A, Sibai BM. Acute fatty liver of pregnancy: an experience in the diagnosis and management of fourteen cases. Am J Obstet Gynecol. 1994;171:1342–7. 3. Browning MF, Levy HL, Wilkins-Haug LE, Larson C, Shih VE. Fetal fatty acid oxidation defects and maternal liver disease in pregnancy. Obstet Gynecol. 2006;107(1):115–20. 4. Ibdah JA, Bennett MJ, Rinaldo P, Zhao Y, Gibson B, Sims HF, Strauss AW. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med. 1999;340:1723–31. 5. Gracia PV, Lavergne JA. Acute fatty liver of pregnancy. Int J Gynaecol Obstet. 2001;72:193–5. 6. Liu G, Shang X, Yuan B, Han C, Wang Y. Acute fatty liver of pregnancy: analysis on the diagnosis and treatment of 15 cases. J Reprod Med. 2016;61:282–6. 7. Pereira SP, O’Donohue J, Wendon J, Williams R. Maternal and perinatal outcome in severe pregnancy-related liver disease. Hepatology. 1997;26:1258–62. 8. Baldwin GS. Do NSAIDs contribute to acute fatty liver of pregnancy? Med Hypotheses. 2000;54:846–9. 9. Castro MA, Fassett MJ, Reynolds TB, Shaw KJ, Goodwin TM. Reversible peripartum liver failure: a new perspective on the diagnosis, treatment, and cause of acute fatty liver of pregnancy, based on 28 consecutive cases. Am J Obstet Gynecol. 1999;181:389–95.

5

Adrenal Insufficiency Catherine Traill and Stephen H. Halpern

Presentation and Symptoms Definition Adrenal insufficiency (AI) is a clinical syndrome in which the adrenal glands do not produce adequate amounts of glucocorticoids and possibly mineralocorticoids.

Symptoms The symptoms of AI are non-specific and may include excessive fatigue, malaise, weight loss, nausea and vomiting, abdominal pain, orthostatic hypotension, syncope, and hyperpigmentation of skin creases and mucosa [1]. Biochemical abnormalities such as hyponatremia, hyperkalemia, hypercalcemia, and hypoglycemia may also be seen.

Incidence Adrenal insufficiency is relatively rare in pregnancy, with a reported incidence of 1 in 3000 births from a series of 15,700 deliveries over a 12-year period [2]. The incidence of AI has been rising over the past few years due to the increase in autoimmune disorders [3].

Classification

1. Primary AI—arises from the direct destruction of the adrenal cortex. This results in both glucocorticoid and mineralocorticoid deficiencies. (a) Autoimmune (Addison’s disease) accounts for 70–90% of AI in the United States. It may be associated with other autoimmune conditions, including type 1 diabetes, hypothyroidism, hypoparathyroidism, and pernicious anemia. (b) Tuberculosis is the second most common cause worldwide. (c) Rarer causes include HIV, bilateral adrenal hemorrhage, metastatic malignancies, and drugs such as ketoconazole and etomidate. 2. Secondary AI—arises from a lack of adrenocorticotropic hormone (ACTH) which results in glucocorticoid deficiency only. (a) Chronic use of exogenous glucocorticoids is the most common cause. This results in suppression of both corticotrophin-releasing hormone (CRH) production in the hypothalamus and ACTH production in the pituitary. This may lead to functional adrenal gland atrophy in severely affected patients. (b) Other causes may arise from the hypothalamus, which results in decreased CRH, or the pituitary, which results in decreased ACTH. Such causes include pituitary adenomas, craniopharyngiomas, Sheehan’s syndrome, lymphocytic hypophysitis, pituitary apoplexy, hypothalamic tumors, and treatments associated with such tumors including surgical resection and cranial irradiation.

Adrenal insufficiency may be primary or secondary.

Interaction with Pregnancy Effects of Pregnancy on Adrenal Insufficiency C. Traill, M.D. • S. H. Halpern, M.D. (*) Department of Anesthesia, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON, Canada e-mail: [email protected]

1. During normal pregnancy the hypothalamic-pituitary-­ adrenal (HPA) axis is activated, resulting in increased cortisol secretion. Cortisol levels rise to about three times

© Springer International Publishing AG, part of Springer Nature 2018 S. K. W. Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_5

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C. Traill and S. H. Halpern

nonpregnant levels by midpregnancy. Plasma ACTH levels have been reported as remaining normal, increasing or decreasing in early pregnancy, or rising gradually after that to two times nonpregnant levels in a similar time frame to the cortisol rise [3] (see Fig. 5.1). 2. The ACTH increase seen may be attributed to placental synthesis of ACTH and CRH, resetting of the pituitary feedback mechanism for cortisol to a higher set point, or enhanced response of the pituitary to CRH [4]. 3. Despite the increase in ACTH and cortisol during pregnancy, the circadian rhythm is maintained [3]. 4. The increase in maternal cortisol secretion is important to fetal growth, development, and organ maturation. 5. During normal pregnancy, unrecognized maternal AI may be masked by transplacental passage of cortisol from fetus to mother, primarily during the third trimester. Consequently AI may only become apparent during the stress of labor, delivery, or postpartum [3]. 6. The stress of labor and delivery can precipitate an Addisonian crisis, the features of which are profound hypotension, hypothermia, altered level of consciousness, cardiovascular collapse, metabolic acidosis, hyponatremia, and hyperkalemia [5]. 7. In pregnant patients with primary AI, cortisol secretion does not increase during pregnancy. The increase in 70

60

400 40

)( , )

300 200 80

30

70 60 50

20

)( )

ACTH (pg/mL) (

Cortisol (µg/dL) (

50

500

40 30

10

20 10 0

10

20

30

40

Weeks of gestation

Fig. 5.1  ACTH and cortisol levels throughout gestation. Courtesy of Springer, Yuen et al. (2013), Adrenal insufficiency in pregnancy: challenging issues in diagnosis and management. Endocrine, 44 (2), 283–292

ACTH secretion results in increased skin and mucous membrane pigmentation.

Effects of Adrenal Insufficiency on Pregnancy 1. With the availability of synthetic steroids, maternal mortality rates have dropped significantly, with no maternal deaths reported since the 1950s [1]. 2. If left untreated, intrauterine growth restriction and low birth weight occur frequently [4]. 3. There have been multiple reports of intrauterine death occurring with AI in pregnancy; however many of these cases occurred in unrecognized cases or before the availability of modern glucocorticoid replacement regimens [4]. 4. There appears to be no increased risk of abortion from AI alone, when treated appropriately [4].

Medical Management Testing and Diagnosis Diagnosis of AI in pregnancy can be difficult and requires a high index of suspicion. Confirmation of diagnosis involves three stages [6]: 1. Demonstration of inappropriately low serum cortisol levels 2. Measurement of plasma ACTH which will be elevated in primary AI or normal or low in secondary AI 3. Identification of a treatable cause

Medical Management 1. Hydrocortisone may be used for glucocorticoid replacement during pregnancy at a dose of 20–30 mg/day. Equivalent doses of prednisone or dexamethasone are also appropriate. Two thirds are given upon awakening, with the remaining third to be given in the afternoon, in keeping with the normal circadian rhythm of cortisol [6]. 2. In the case of primary AI, mineralocorticoid replacement with fludrocortisone 0.05–0.2 mg/day is appropriate, dependent upon blood pressure and serum potassium levels [6]. 3. Plasma ACTH and cortisol levels cannot be used to adjust dosing of glucocorticoid replacement. 4. Glucocorticoid treatment is not associated with teratogenicity, maternal infections, fetal loss, or poor wound healing [6].

5  Adrenal Insufficiency

5. In undiagnosed cases of AI, the most critical periods are during the first trimester, when symptoms of adrenal crisis can be mistaken for pregnancy-induced emesis, and during the stress of labor and delivery. Such stress can precipitate an Addisonian crisis which can be life-­threatening [6]. 6. In cases of adrenal crises, prompt glucocorticoid therapy should be administered in the form of 100–200 mg of intravenous hydrocortisone, followed by 50–100 mg every 6–8 h. Intravenous dextrose 5% and potassium supplementation may also be required. Fludrocortisone is not indicated during an acute event due to the mineralocorticoid-­ like properties of high-dose glucocorticoids [6]. Once the patient is able to tolerate fluids, oral glucocorticoid replacement can begin.

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intravenous hydrocortisone before surgery begins and then 50–100 mg every 6–8 h postpartum. The dose should be tapered to maintenance within 3 days [6]. 2. If general anesthesia is required, the patient should be optimized preoperatively to avoid the risk of significant myocardial depression that may occur with induction and maintenance of general anesthesia. 3. Adequate intravenous hydration is required.

Postoperative Management

Medication

1. Patients should recommence their prepregnancy doses of glucocorticoid with or without mineralocorticoid once they are able to tolerate oral intake. This can occur immediately after delivery in uncomplicated cases [2]. 2. In a minority of cases, stress doses of glucocorticoids may be necessary if the patient is in pain or while recovering from surgery [6].

1. At the onset of labor, intravenous hydrocortisone should be administered at a dose of 50–100 mg and repeated every 6–8 h until delivery.

References

Anesthetic Management

Neuraxial Analgesia 1. Vaginal delivery is the best option for delivery if there are no symptoms of undertreatment. 2. There are no contraindications to neuraxial analgesia in these patients. Given labor and delivery are considered stressful events, appropriate analgesia may help to alleviate the stress response to labor.

Anesthesia for Cesarean Delivery 1. If Cesarean section is indicated, the patient should receive supplemental corticosteroids in the form of 100 mg of

1. Otta CF, de Mereshian PS, Iraci GS, de Pruneda MRO. Pregnancies associated with primary adrenal insufficiency. Fertil Steril. 2008;90(4):1199 e17–20. 2. Albert E, Dalaker K, Jorde R, Berge LN. Addison’s disease and pregnancy. Acta Obstet Gynecol Scand. 1989;68(2):185–7. 3. Ambrosi B, Barbetta L, Morricone L. Diagnosis and management of Addison’s disease during pregnancy. J Endocrinol Invest. 2003;26(7):698–702. 4. Lindsay JR, Nieman LK. The hypothalamic-pituitary-adrenal axis in pregnancy: challenges in disease protection and treatment. Endocr Rev. 2005;26(6):775–99. 5. Seaward PG, Guidozzi F, Sonnendecker EW. Addisonian crisis in pregnancy. Case report. Br J Obstet Gynaecol. 1989;96(11):1348–50. 6. Yuen KC, Chong LE, Koch CA. Adrenal insufficiency in pregnancy: challenging issues in diagnosis and management. Endocrine. 2013;44(2):283–92.

6

Amphetamines and Other Stimulant Use Curtis L. Baysinger

Epidemiology

Pharmacology and Physiology of Stimulants

1. Nonmedical use of prescription stimulants accounts for 2/3 of the 1.6 million people above the age of 12 currently abusing amphetamines or similar stimulants. Nearly 600,000 (approximately 1/3) were estimated to be current users of methamphetamine [1]. These percentages are similar to those reported over the interval 2002–2013 [1]. 2. Methamphetamine abuse among pregnant women increased from 8% of federally funded admissions in 1994 to 24% in 2006, with an estimated 5.2% of high-risk pregnant women estimated to have used it during their pregnancy in that later year [2]. The dramatic increases were most probably facilitated by its widespread availability from home laboratories [3]. Although the rate of hospitalization for methamphetamine has doubled since the mid-1990s [4], recent trends suggest a plateauing in new use, maybe reflecting new restrictions on the sale of ingredients used in its illicit manufacture [1]. However, in contrast to other drugs of abuse use of which significantly decline during pregnancy, methamphetamine remains high up until delivery [5]. 3. MDMA (3,4-methylenedioxymethamphetamine, ecstasy) use during pregnancy is difficult to determine. Approximately 900,000 persons overall are estimated to begin use each year in the USA, with approximately 500,000 having used in the last month; there is approximately equal usage by gender. The number of women who abuse the drug while pregnant is unclear [1].

1. Amphetamine-like drugs exhibit effects similar to that of cocaine by increasing dopamine, serotonin, and norepinephrine availability to postsynaptic neurons, but because amphetamines can act as false transmitters, dopamine release is further increased than that by reuptake inhibition alone and is thus higher than that for cocaine [6]. The activation of dopaminergic neurons with the ventral tegmental areas of the mesolimbic system is similar to that of cocaine and, like cocaine, is responsible for the drugseeking behavior. 2. MDMA is a derivative of methamphetamine and thus has similar effects on dopaminergic and postsynaptic sympathetic neurons but affects serotonin (5HT) reuptake in serotonergic neurons to a much greater degree [7]. 3. Amphetamine and methamphetamine can be consumed by snorting, smoking, intravenous injection, or orally. When methamphetamine is smoked, the euphoric effects are intense and of moderate duration, thus making it highly addictive compared to other drugs of abuse [2]. Its greater potency and longer half-life may also explain its greater addictive potential compared to amphetamine [6]. MDMA is primarily consumed orally. 4. Sympathetic nervous system becomes activated, similar to that with cocaine ingestion. Tachycardia, hypertension with labile blood pressure, arrhythmias, increases in systemic vascular resistance, and fever can be expected in the acutely intoxicated women, and seizures combined with hypertension may make differentiation from eclampsia difficult as in the cocaine-intoxicated parturient. However, amphetamines lack the local anesthetic effect of cocaine, and their inhibition of monoamine oxidase activity may prolong catecholamine effect longer than that due to cocaine ingestion [6]. Serotonergic toxicity with MDMA use can lead to significant hyperthermia, and death has been reported in one pregnant woman [8].

C. L. Baysinger, M.D. Department of Anesthesiology, Vanderbilt University Medical Center, Vanderbilt University Hospital, Nashville, TN, USA e-mail: [email protected]

© Springer International Publishing AG, part of Springer Nature 2018 S. K. W. Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_6

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5. Chronic use leads to central dopamine depletion, the consequences of which are compulsive drug-seeking behavior, anxiety, confusion, and violent behavior [6, 9]. 6. Chronic use causes other end-organ damage. Weight loss and severe dental damage (“meth mouth”) accompany chronic methamphetamine use [6, 9]. The psychotic features of longtime amphetamine abuse may not recover fully after long periods of abstinence, and neurologic damage of stroke and psychosis may be higher among methamphetamine abusers than for cocaine and other amphetamine-like drugs [6, 9].

Effects on Pregnancy and the Fetus 1. Two differing populations abuse stimulants during pregnancy; one abuses drugs prescribed for attention deficit and sleep disorders (poorly studied) and the other which uses illicit drugs (better studied) [10]. However, stimulants have been less well studied than alcohol, cocaine, and opioids [1]. Outcome data is confounded by maternal use of other substances [11]. One recent study noted that 78% of women who used methamphetamine smoked, 14% drank alcohol, and nearly ¼ tested positive for other illicit drugs [4]. 2. Adverse obstetric outcomes are increased. The risk for small for gestational age babies is increased (odds ratio 3.5), with smaller head circumference, weight, and height [12], and observational studies of neonatal behavior report sleep disruption, feeding difficulties, and muscle tone abnormality [6]. However, very few of these infants required treatment [12], and the effectiveness of treatments is not known [10]. Although theoretical concerns over the effects of decreased uterine blood flow created by catecholamine excess have been voiced, whether rates of placental abruption, preterm birth, acute delivery for fetal reasons in the non-intoxicated mother, or preeclampsia are higher is unclear [11]. 3. Stimulant abuse may lead to long-term neonatal and childhood consequences but may be less than that associated with other abused substances. Small studies comparing children exposed to in utero amphetamines vs. those who were not show greater attention deficits, and decreased memory and visual acuity, but similar motor and nonverbal intelligence scores later in childhood [13]. Recruitment of more brain compensatory pathways has been demonstrated by magnetic resonance imaging in children born of methamphetamine-abusing women compared to children whose mothers abused other drugs [13]. Other birth defects do not appear to occur with greater frequency; however, babies are typically small for gestational age [4]. Chronic exposure to MDMA may cause more significant long-term consequences than for other

C. L. Baysinger

simulants as serotonin plays a key role in fetal brain development more than other neurotransmitters [8].

Obstetric and Medical Management 1. Acute intoxication with hypertension can be treated with labetalol and hydralazine. The hyperthermic mother can be actively cooled and should receive seizure prophylaxis with benzodiazepines. The fetus should be monitored [14]. 2. Effective treatments for long-term chronic abuse are unknown [7], but all women should have brief interventions and support therapy and offered treatment regardless of pregnancy status, with inpatient residential programs being recommended [3, 7]. Pharmacologic treatments have not been shown to be effective, but amantadine and propanol may help with severe withdrawal symptoms [6, 7]. 3. Intrapartum and postpartum management should be similar to that of other drugs of abuse with increased fetal surveillance and increased frequency of non-stress and biophysical profiling [3, 14]. Plans for delivery should be based on the same indications than in non-stimulant-­ abusing women [3, 14]. Urine drugs screens should be used with patient consent [3, 14]. Awareness of what to do with positive results prescribed by laws in a given practice location is required. Meconium testing with maternal permission may be of value [3, 14]. Women who are actively using methamphetamines and other illicit stimulants should not breastfeed [15].

Anesthetic Management 1. Recommendations for anesthetic management are similar to those for cocaine abuse. Neuraxial anesthesia/analgesia may be accompanied by more frequent and more difficult to treat hypotension due to catecholamine depletion in the chronic non-intoxicated abuser [16, 17], although the frequency with which this might occur has not been determined. Similar to cocaine, the use of the direct-acting sympathomimetic phenylephrine may be more efficacious than an indirect-acting agonist such as ephedrine [16, 17]. An increased risk for significant cardiovascular events may be more likely in the acutely intoxicated patient [16, 17], although the magnitude of that risk is not known. 2. Temperature monitoring of the patient acutely intoxicated with MDMA may be more critical than with other stimulants [16, 17]. 3. General anesthesia may be required for urgent delivery. Acute amphetamine intoxication in animals increases the MAC for volatile agents, while chronic administration and withdrawal decrease MAC [18]. Direct l­aryngoscopy

6  Amphetamines and Other Stimulant Use

would be expected to increase the hypertension and tachycardia typically present in the intoxicated patient, so measures to blunt those responses (pretreatment with labetalol, hydralazine, nitroglycerine, nicardipine, etc.) should be available. Dentition may be poor and interfere with laryngoscopy. Similar to management of the cocaine-­intoxicated patient, avoidance of catecholamine-­ sensitizing inhalational anesthetics would be wise [19]. Others suggest avoidance of succinylcholine in the acutely intoxicated patient out of concerns of a hyperkalemic response to its use [19]. 4 . Stimulant-abusing patients may have higher analgesia requirements both during labor and post cesarean delivery, but probably not as great as in the opioid.

References 1. Substance Abuse and Mental Health Services Administration. Results from the 2013 National Survey on Drug Use and Health: summary of national findings. NSDUH series H-48, HHS publication no. (SMA) 14-4863. Rockville: Substance Abuse and Mental Health Services Administration; 2014. 2. Arria AM, Derauf C, Lagasse LL, et al. Methamphetamine and other substance use during pregnancy: preliminary estimates from the Infant Development, Environment, and Lifestyle (IDEAL) study. Matern Child Health J. 2006;10:203–302. 3. American College of Obstetricians and Gynecologists. Committee opinion number 479: methamphetamine abuse in women of reproductive age. Obstet Gynecol. 2011;117:751–5. 4. Good MM, Solt I, Acuna JG, et al. Methamphetamine abuse during pregnancy: maternal and neonatal implications. Obstet Gynecol. 2010; 116:330–4. 5. Della Grotta S, LaGasse LL, Arria AM, et al. Patterns of methamphetamine use during pregnancy: results from the Infant

23 Development, Environment, and Lifestyle (IDEAL) study. Matern Child Health J. 2010;14:519–27. 6. Hyman SE. Addiction to cocaine and amphetamine. Neuron. 1996;16:901–4. 7. Rayburn WF, Bogenschutz MP. Pharmacotherapy for pregnant women with addictions. Am J Obstet Gynecol. 2004;191:1885–97. 8. Singer LT, Moore DG, Meeyoung O, et al. One year outcomes of prenatal exposure to MDMA and other recreational drugs. Pediatrics. 2012;130:407–13. 9. Reynolds EW, Bada HS. Pharmacology of drugs of abuse. Obstet Gynecol Clin North Am. 2003;30:501–22. 10. Frost EAM, Gist RS, Adriano E. Drugs, alcohol, pregnancy, and the fetal alcohol syndrome. Int Anesthesiol Clin. 2011;49:119–33. 11. American College of Obstetricians and Gynecologists. ACOG Committee opinion no. 422: at-risk drinking and illicit drug use: ethical issues in obstetric and gynecologic practice. Obstet Gynecol. 2008;112:1449–60. 12. Smith LM, LaGasse LL, Derauf C, et al. Prenatal methamphetamine use and neonatal neurobehavioral outcome. Neurotoxicol Teratol. 2008;24:17–23. 13. Lu HL, Johnson A, O’Hre ED, et al. Effects of prenatal methamphetamine exposure on verbal memory revealed with functional magnetic resonance imaging. J Dev Behav Pediatr. 2009;30:185–92. 14. McLafferty LP, Becker M, Dresner N, et al. Guidelines for the management of pregnant women with substance abuse disorders. Psychosomatics. 2016;57:115–30. 15. D’Apoito K. Breastfeeding and substance abuse. Clin Obstet Gynecol. 2013;56:202–11. 16. Hanzawa S, Nemoto M, Etoh S, et al. A case of amphetamine-­ induced down-regulation of the beta-adrenoceptor. Mausi. 2001;50:1242–5. 17. Kuczkowski KM. Anesthetic implications of drug abuse in pregnancy. J Clin Anesth. 2003;15:382–94. 18. Johnston RR, Way WL, Miller RD. Alteration of anesthetic requirement by amphetamine. Anesthesiology. 1972;36:357–63. 19. Sullivan JT. Substance abuse and the drug addicted mother. In: Suresh MS, Segal S, Preston RL, Fernando R, Mason CL, editors. Shnider and Levinson’s anesthesia for obstetrics. 5th ed. Baltimore: Wolters Kluwer; 2013. p. 683–98.

7

Amyotrophic Lateral Sclerosis Christopher K. Der and Jie Zhou

Background

Symptoms

1. Amyotrophic lateral sclerosis (ALS) is a rapidly progressive degeneration of upper and lower motor neurons, resulting in muscle weakness and atrophy. This degeneration of motor neurons occurs in the cerebral cortex, brain stem, and spinal cord. 2. The pathogenesis has not been fully elucidated. The current understanding of ALS includes isolation of multiple genes, which are found in familial as well as sporadic cases. The familial form is autosomal dominant, X-linked recessive, or mitochondrial and sporadic cases account for the majority of diseases. Many factors have been identified as contributing to the pathophysiology of ALS including genetic; RNA processing; protein misfolding, aggregation, and degradation; mitochondrial dysfunction; dysfunction of glutamatergic signaling; oxidative stress; impaired axoplasmic flow; neuroinflammation; and many others [1]. Genetic and environmental factors both play a role: clinical symptoms may develop after repeated exposure to various triggers [2, 3]. 3. Median survival is 3–5 years [2]. 4. Diagnosis is based on clinical findings according to the El Escorial criteria. Lower and upper motor neuron degeneration is based on clinical or electrophysiologic examinations in several regions after excluding other diseases [4].

1. There are many symptoms depending on specific motor neuron involvement. Asymmetric weakness and atrophy typically occur in the limb, thoracic, and abdominal muscles. Cramps, fasciculations, gait disturbances, facial diparesis, spasticity, and hyperreflexia occur. 2. Bulbar involvement may lead to dysarthria, dysphagia, chewing difficulties, hypersialorrhea, and palatal weakness. Other concerns for the anesthesiologist include pharyngeal secretions, trismus, jaw clonus, and laryngospasm [6]. 3. Pseudobulbar symptoms appear as altering extreme emotional states [6]. 4. At the end stage, these patients typically are bed bound and often require mechanical ventilator support and gastroenteral support [5].

Incidence ALS appears to have an incidence approaching 1–3:100,000 over the age of 15, increasing with age and peaking at an older age. Men are affected more than women. Thus, ALS during pregnancy is rare [1, 2, 5]. C. K. Der, M.D. • J. Zhou, M.D., M.S., M.B.A. (*) Department of Anesthesiology, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA e-mail: [email protected]

Interaction with Pregnancy Effect of Pregnancy on ALS 1. Because of the rarity of ALS during pregnancy, data is mostly provided through case reports. 2. Of interest, many cases have had their initial presentation during pregnancy. This could mean that pregnancy unmasks a previously quiescent disease, and this could be due to the hormonal effects, neuroinflammation, and cytokine activation during pregnancy [3, 7]. 3. It is not known if disease progression is affected by pregnancy. However, most reports indicate that ALS does not adversely affect the course of the disease, especially when not in the late stages [7]. 4. However, parturients with severe respiratory compromise appear to experience worsening of function. This is largely due to the physiologic changes of pregnancy with significant increased minute ventilation based on an increased tidal volume and diaphragmatic breathing. The latter is compromised in parturients with pulmonary

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involvement [7]. The increasing gravid uterine size, displacement of the stomach, and elevation of diaphragm also contribute to worsening of already compromised respiratory mechanics [8, 9].

Effect of ALS on Pregnancy 1. Because of the rapidly progressive nature of the disease, symptomatology may dictate the route of delivery. 2. However, most parturients tolerate labor and delivery well. ALS does not affect the uterus. Most patients, even those with pelvic floor dysfunction, enjoy vaginal deliveries [2, 7, 8]. 3. Forceps or vacuum delivery may be helpful in assisting with a vaginal delivery in more compromised parturients. However, in parturients with impending respiratory failure, this may not be possible. Thus, cesarean delivery may be necessary for worsening maternal status or for obstetric indications [5, 7]. 4. There appear to be no adverse obstetrical or fetal affects, which occur in parturients with ALS [2, 7, 10].

Management Medical Management 1. Medical management is the mainstay of treatment for symptoms. There is no cure. 2. Riluzole is an FDA-approved medication (class C) for ALS. The drug may slow the progression of symptoms in some patients, by reducing glutamate levels. Despite preclinical studies showing teratogenic effects at higher than clinically normal doses, the medication has been used during pregnancy. There are only a few case reports, one without adverse neonatal effects and one with a small atrial communication and patent ductus arteriosus at 6 days of life. More literature is necessary to determine the safety profile of this intervention [2, 10]. 3. There is one report of a successful bilateral autologous stem cell transplant into the frontal motor cortex 15 months after delivery in a parturient who developed ALS during the second trimester of pregnancy. This resulted in stabilization of disease and delayed progression [11]. 4. Other medications attempt to control and provide relief from symptoms such as constipation, spasticity, depression, pain, salivation, and sleep disorders. 5. Consideration should be given to thromboprophylaxis. Weakness and immobility may increase the risk of thrombotic events. 6. Treatment for life-threatening symptoms is supportive. Mechanical ventilation may be necessary.

Anesthetic Management 1. The severity of the disease will likely dictate management. Antenatal assessment of weakness and cardiopulmonary status is critical. The potential need for postoperative ventilation should be discussed. 2. There is no contraindication to neuraxial analgesia and anesthesia for labor and cesarean delivery. There is little literature on the anesthetic management in parturients with ALS. However, if a parturient has respiratory disease, epidural analgesia can minimize the work of breathing during labor and delivery. In addition, further compromise from a general anesthetic could be deleterious. In the setting of compromised respiratory mechanics, titration of epidural local anesthetic to the desired level has been advocated. Intrathecal anesthesia has also been used successfully. However, if there is concern about a high level adversely affecting pulmonary function, a combined spinal-epidural may be preferable [5, 12]. 3. Some patients will benefit from noninvasive positive pressure ventilation (NIPPV) in the intraoperative period [6]. 4. Some advocate arterial blood gas monitoring and pulse oximetry during labor. Arterial blood gases may assist in recognizing failing respiratory mechanics due to fatigue during the second stage of labor or from a high level of regional blockade [7]. 5. The risks of general anesthesia may far outweigh those of regional anesthesia. General anesthesia could lead to a greater chance of aspiration and respiratory decompensation, with the need for prolonged mechanical ventilation. There are many other risks that may complicate anesthetic management including trismus, laryngospasm, airway secretions, and bulbar dysfunction [6]. (a) Short-acting anesthetics and analgesics, such as remifentanil, should be used. (b) Similarly volatile anesthetics, such as desflurane and sevoflurane, with lower lipid solubility should be considered. (c) Succinylcholine should be avoided in patients with advanced disease as life-threatening hyperkalemia has been described. (d) Non-depolarizing neuromuscular blocks should also be used with caution. They may antagonize postsynaptic receptors, preventing the binding of acetylcholine. Prolonged weakness has been described. Twitch monitoring is imperative [6]. (e) Prolonged mechanical ventilation may be necessary. 6. Continuation of the epidural analgesia in the postpartum period may be helpful, particularly in those with respiratory compromise. Systemic opioids may further interfere with respiratory mechanics.

7  Amyotrophic Lateral Sclerosis

7. Pulmonary function testing may be beneficial in determining the need for noninvasive positive pressure ventilation (NIPPV) in the postpartum period [6].

References 1. Morgan S, Orrell RW. Pathogenesis of amyotrophic lateral sclerosis. Br Med Bull. 2016;119(1):87–98. 2. Kawamichi Y, Makino Y, Matsuda Y, Miyazaki K, Uchiyama S, Ohta H. Riluzole use during pregnancy in a patient with amyotrophic lateral sclerosis: a case report. J Int Med Res. 2010;38(2):720–6. 3. Lunetta C, Sansone VA, Penco S, Mosca L, Tarlarini C, Avemaria F, et al. Amyotrophic lateral sclerosis in pregnancy is associated with a vascular endothelial growth factor promoter genotype. Eur J Neurol. 2014;21(4):594–8. 4. Al-Chalabi A, Hardiman O, Kiernan MC, Chiò A, Rix-Brooks B, van den Berg LH. Amyotrophic lateral sclerosis: moving towards a new classification system. Lancet Neurol. 2016;15(11):1182–94. 5. Sarafov S, Doitchinova M, Karagiozova Z, Slancheva B, Dengler R, Petri S, et al. Two consecutive pregnancies in early and late

27 stage of amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2009;10(5–6):483–6. 6. Prabhakar A, Owen CP, Kaye AD. Anesthetic management of the patient with amyotrophic lateral sclerosis. J Anesth. 2013;27(6):909–18. 7. Chiò A, Calvo A, Di Vito N, Vercellino M, Ghiglione P, Terreni AA, et al. Amyotrophic lateral sclerosis associated with pregnancy: report of four new cases and review of the literature. Amyotroph Lateral Scler Other Motor Neuron Disord. 2009;4(1):45–8. 8. Tyagi A, Sweeney BJ, Connolly S. Amyotrophic lateral sclerosis associated with pregnancy. Neurol India. 2001;49(4):413–4. 9. Lupo VR, Rusterholz JH, Reichert JA, Hanson SA. Amyotrophic lateral sclerosis in pregnancy. Obstet Gynecol. 1993;82(4):682. 10. Scalco RS, Vieira MC, da Cunha Filho EV, Lago EG, da Silva IG, Becker J. Amyotrophic lateral sclerosis and riluzole use during pregnancy: a case report. Amyotroph Lateral Scler. 2012;13(5):471–2. 11. Martínez HR, Marioni SS, Ocañas CEE, Garza MTG, Moreno-­ Cuevas JE. Amyotrophic lateral sclerosis in pregnancy: clinical outcome during the post-partum period after stem cell transplantation into the frontal motor cortex. Cytotherapy. 2014;16(3):402–5. 12. Hobaika AB, Neves BS. Combined spinal-epidural block in a patient with amyotrophic lateral sclerosis: case report. Rev Bras Anestesiol. 2009;59(2):206–9.

8

Anticoagulation Jeanette R. Bauchat and Joseph B. Bavaro

Coagulation and Pregnancy 1. Pregnancy is a prothrombotic state, with all components of Virchow’s triad activated during pregnancy, labor, and delivery [1]. (a) Pregnancy is a hypercoagulable state with upregulation of most clotting factors and decreased fibrinolytic activity [2–4]. (b) Pregnant women are at increased risk for venous stasis due to vena caval compression by the gravid uterus and decreased activity levels, particularly late in pregnancy [5, 6]. (c) Operative (vaginal or cesarean) deliveries and placental separation from the uterus can cause vascular damage [2]. 2. Pregnant women are at increased risk for thromboembolic phenomena [2]. (a) Approximately 1 in 500 to 1 in 2000 pregnancies are complicated by venous thromboembolism (VTE) in the United States [1, 2, 7, 8]. (b) VTE was the sixth leading cause of maternal mortality in the United States from 2006 to 2010, accounting for nearly 10% of all pregnancy-related deaths [9]. (c) The risk of VTE is increased 5- to 50-fold during pregnancy, compared with prepregnancy risk, and increases further to 60-fold in the postpartum period. The risk returns to baseline levels by 18 weeks postpartum [7, 10]. (d) Additional risk factors for VTE during pregnancy include black race (1.5× risk compared to white)

J. R. Bauchat, M.D., M.S. (*) Division of Obstetric Anesthesiology, Vanderbilt University Medical Center, Nashville, TN, USA e-mail: [email protected] J. B. Bavaro, M.D., M.S. Department of Anesthesiology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA e-mail: [email protected]

[11], diabetes mellitus (either pregestational or gestational) [12], multiple gestations [13], obesity [14], age >35 [14], and a history of an inherited thrombophilia (see below).

I ndications for Anticoagulant Medication Use During Pregnancy 1. Anticoagulant medications may be prescribed to pregnant patients for a variety of indications. The American College of Obstetricians and Gynecologists (ACOG) has issued several practice bulletins to address or indicate which pregnant women should be anticoagulated [15, 16]. (a) Women with a personal history of VTE are often anticoagulated during pregnancy, especially if VTE was unprovoked (i.e., not due to trauma, prolonged immobility, oral contraceptive use, etc.). (b) Even in the absence of a history of VTE, pregnant women with high-risk thrombophilias may also be anticoagulated. • High-risk thrombophilias include antithrombin III deficiency, prothrombin gene mutation homozygotes, factor V Leiden homozygotes, compound heterozygotes (i.e., heterozygous for both prothrombin gene mutation and factor V Leiden), and the presence of antiphospholipid antibodies. • Low-risk thrombophilias include prothrombin gene heterozygotes, factor V Leiden heterozygotes, protein S deficiency, and protein C deficiency. (c) Additional indications for anticoagulation in pregnancy include atrial fibrillation, prosthetic heart valves, or cerebral venous thrombosis [17, 18]. (d) In pregnant women at high-risk for preeclampsia (chronic hypertension, previous preterm preeclampsia, and diabetes), the US Preventive Services Task Force (USPSTF) recommends starting low-dose (81mg po daily) aspirin early in pregnancy to reduce this risk [19, 20]. This may further complicate timing

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and safety of neuraxial anesthesia if patients remain on dual therapy at the time of delivery. 2. ACOG provides guidelines for dosing regimens of anticoagulant medications [15, 16]. These recommendations vary, depending on whether anticoagulation is prophylactic or therapeutic. 3. When safe and feasible, ACOG recommends transitioning from low molecular weight heparin to unfractionated heparin at 36 weeks gestational age. Unfractionated heparin has a shorter duration of action that may facilitate placement of neuraxial anesthesia and reduce the risk of obstetric hemorrhage [16].

Anticoagulant Medications 1. Antiplatelet agents (a) Aspirin irreversibly acetylates the platelet enzyme cyclooxygenase 1 (COX-1), inhibiting thromboxane A2 and thus preventing platelet aggregation and local vasoconstriction [21]. • Platelets return to baseline function 5 days after aspirin cessation and only platelet transfusion can restore platelet function if rapid reversal is needed [21]. • Aspirin is contraindicated in the third trimester of pregnancy due to fetal morbidity such as premature closure of the ductus arteriosus and renal dysfunction and possible prolongation of bleeding time in the mother and fetus [22]. (b) Adenosine 5′-diphosphate (ADP) inhibitors (clopidogrel, ticlodipine) block the P2Y12 receptor on the surface of platelets, thereby preventing the binding of ADP to the receptor and thus inhibiting platelet aggregation. • Platelet inhibition lasts 5 days after the discontinuation of these drugs and there is no reversal agent. Pregnant woman receiving these agents would be at high risk of obstetric hemorrhage, and platelet transfusion would be the only way to reverse its effects [21]. • ADP inhibitors are rarely used in pregnancy since they are primarily indicated for recent coronary artery stent placement or to prevent cerebrovascular accidents [23]. 2. Heparinoid agents are considered first-line anticoagulation agents in pregnant or lactating women as they neither cross the placenta nor are excreted in breast milk [24]. (a) Unfractionated heparin (UFH) binds antithrombin III to form a complex that inactivates coagulation factors IIa (thrombin), IXa, Xa, XIa, and XIIa. • UFH is best monitored by aPTT and can be reversed by protamine administration [21]. • The half-life of UFH is relatively short (1–2 h), although this may be dose-dependent and is prolonged by renal and hepatic dysfunction [25]. (b) Low molecular weight heparins (dalteparin, enoxaparin, nadroparin, tinzaparin) bind antithrombin III and inhibit

J. R. Bauchat and J. B. Bavaro

coagulation factors. They have a reduced ability to inactivate thrombin but enhanced anti-factor Xa activity. • LMWH can only be partially reversed with protamine and factor VIIa [21]. • Anti-factor Xa levels may be used to monitor therapeutic efficacy in pregnant women [26]. 3. Vitamin K antagonists (warfarin) prevent synthesis of clotting factors II, VII, IX, and X as well as anticlotting proteins C and S within 72–96 h of dosing [27]. Warfarin is teratogenic and is not used in pregnancy. 4. Non-vitamin K oral anticoagulants or novel oral anticoagulants (NOACs) such as thrombin II inhibitors and factor Xa inhibitors have fast onset, short half-lives, and predictable pharmacokinetics requiring no monitoring [21]. (a) Thrombin II inhibitors (dabigatran, argatroban) bind thrombin directly. A normal aPTT ensures no residual effect of dabigatran remains. Recently, the Food and Drug Administration approved the drug, idarucizumab, to reverse dabigatran [28]. (b) Indirect factor Xa inhibitors (fondaparinux) and direct factor Xa inhibitors (apixaban, rivaroxaban, edoxaban) have no reversal agents. The effects of these agents can generally be detected on PT, aPTT, and factor Xa assays, but there are no therapeutic ranges established [21, 29]. 5. Few studies have been conducted using these agents in pregnancy, but they are typically reserved for pregnant patients who have a contraindication to heparinoid drugs or failed heparin therapy.

 ultidisciplinary Approach to Caring M for the Anticoagulated Parturient 1. Pregnant women on anticoagulation should have a consultation with an anesthesiologist who practices obstetric anesthesia on a regular basis. The consultation should include the anticoagulation plan to optimize maternal health to minimize the risk of neuraxial analgesic complications, postpartum hemorrhage, and surgical bleeding during cesarean delivery. 2. Team members who need to be included in management discussions include obstetricians or maternal fetal medicine physicians, anesthesiologists, nursing staff, and, depending on the circumstance for anticoagulation, a hematologist or cardiologist (Table 8.1).

 afety and Timing of Neuraxial Techniques S in the Anticoagulated Parturient 1. Anticoagulation is a relative contraindication for placement of neuraxial anesthesia due to the risk of a spinal or epidural hematoma. The type and timing of cessation of

8 Anticoagulation

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Table 8.1  Considerations for anesthesiologist consultation in the anticoagulated pregnant woman Maternal health optimization

Neuraxial labor analgesia considerations

Risk of postpartum or operative hemorrhage

Questions What is the indication for anticoagulation? Is it safe to stop anticoagulation? If it is safe to stop temporarily, when should the anticoagulant drug be held? Is there an alternative anticoagulant with a shorter half-life or that has a viable reversal agent available? What other forms of thromboembolism prophylaxis can be used while she is not being medically anticoagulated? What laboratory values are needed to minimize the risk of neuraxial complications? Is it safe to perform a neuraxial technique? Is it safe to using a reversal agent and perform a neuraxial technique? What are alternatives to neuraxial labor analgesia? If anticoagulation is needed postpartum, what should the timing be for restarting anticoagulation? What baseline laboratory values are needed to assess the risk of postpartum hemorrhage? Can the anticoagulant drug be reversed in the case of a postpartum hemorrhage or emergent cesarean delivery? What drugs or blood products need to be available to reverse the effects of anticoagulation?

the anticoagulant agent is critical to minimizing complications related to neuraxial techniques. (a) The risk of spinal epidural hematoma formation is extremely low (~1 in 200,000) in pregnant women [30, 31], and there have been no reports of spinal epidural hematoma formation associated with anticoagulant use in parturients. However, in non-obstetric patients, the risk of spinal epidural hematoma formation does appear to be somewhat increased in the setting of concurrent anticoagulation use, but data are lacking to quantify the magnitude of this increased risk [30, 32, 33]. (b) Without prompt treatment (including neurosurgical consultation for possible laminectomy and/or evacuation), severe neurological sequelae including paralysis may result from hematoma formation in the spinal or epidural space [33]. 2 . Anesthetic considerations (a) Coagulation status can be measured via various assays although it remains unclear whether normal values for these tests, in the presence of anticoagulation therapy, mitigate the risk of developing spinal epidural hematoma following neuraxial anesthesia [34–37]. • PT/aPTT • Factor Xa activity

• Platelet count /platelet function assays • TEG/ROTEM • Factor Xa activity • Platelet function assays (b) The American Society of Regional Anesthesia and Pain Medicine (ASRA) has published guidelines for the timing of neuraxial anesthesia in patients who are anticoagulated. The most recent full edition of these recommendations was published in 2010 and is summarized below [37]; however, updated recommendations are expected to be released soon, and the reader should refer to these newer guidelines once they become available. • Antiplatelet agents –– When used alone, NSAIDs or aspirin do not increase the risk of spinal epidural hematoma formation, and neuraxial anesthesia does not need to be delayed in patients only on these medications. –– Use of NSAIDs or aspirin in addition to other anticoagulant medications may increase the risk of bleeding, including spinal epidural hematoma formation. –– ADP inhibitors: Clopidogrel should be discontinued for 7 days prior to a neuraxial procedure. Ticlodipine should be discontinued for 14 days prior to a neuraxial procedure. • Unfractionated heparin –– Patients who have received UFH for >4 days should have a platelet count checked prior to neuraxial blockade, due to the risk of heparin-induced thrombocytopenia (HIT). –– In patients receiving a total daily dose of subcutaneous UFH of ≤10,000 units, there is no contraindication to performing a neuraxial technique. –– Per the 2010 ASRA guidelines, the safety of neuraxial anesthesia in patients receiving >10,000 units of subcutaneous UFH has not been established. Institutional practices vary; however, based on the half-life of subcutaneous heparin of 1–2 h, our institutional practice is to delay placing a neuraxial anesthetic for 12 h after the last heparin dose unless the patient’s aPTT has normalized. –– The 2010 ASRA guidelines do not specifically address the safety of timing of neuraxial anesthesia in parturients receiving intravenous heparin in the obstetric ­population. However, our institutional practice is to delay placement of neuraxial anesthesia until the patient’s aPTT has normalized following discontinuation of intravenous heparin. –– Unfractionated heparin (regardless of dose) may be restarted 1 h after epidural catheter removal, although concern for obstetric hemorrhage may warrant further delay. • Low molecular weight heparins

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–– In patients receiving LMWH for prophylaxis (e.g., 40 mg daily or 30 mg every 12 h), the neuraxial technique should be delayed until 10–12 h after the last LMWH dose. –– In patients receiving LMHW for treatment (e.g., enoxaparin 1 mg/kg every 12 h, or 1.5 mg/kg daily), neuraxial techniques should be delayed for 24 h after the last LMWH dose. –– LMWH may be started a minimum of 4 h following epidural catheter removal or spinal placement. Additionally, prophylactic LMWH should be restarted no sooner than 12 h after epidural catheter placement, and therapeutic LMWH should be restarted no sooner than 24 h after epidural catheter placement.

References 1. Greer IA. Thrombosis in pregnancy: maternal and fetal issues. Lancet. 1999;353(9160):1258–65. 2. Marik PE, Plante LA. Venous thromboembolic disease and pregnancy. N Engl J Med. 2008;359(19):2025–33. 3. McColl MD, Ramsay JE, Tait RC, Walker ID, McCall F, Conkie JA, et al. Risk factors for pregnancy associated venous thromboembolism. Thromb Haemost. 1997;78(4):1183–8. 4. Hellgren M, Blombäck M. Studies on blood coagulation and fibrinolysis in pregnancy, during delivery and in the puerperium. Gynecol Obstet Invest. 1981;12(3):141–54. 5. Gordon MC. Maternal physiology. In: Gabbe SG, Niebyl JR, Simpson JL, editors. Obstetrics: normal and problem pregnancies. 5th ed. Philadelphia: Churchill Livingstone; 2007. p. 55–84. 6. Macklon NS, Greer IA, Bowman AW. An ultrasound study of gestational and postural changes in the deep venous system of the leg in pregnancy. Br J Obstet Gynaecol. 1997;104:191–7. 7. Heit JA, Kobbervig CE, James AH, Petterson TM, Bailey KR, Melton LJ III. Trends in the incidence of venous thromboembolism during pregnancy or postpartum: a 30-year population-based study. Ann Intern Med. 2005;143:697–706. 8. Morris JM, Algert CS, Roberts CL. Incidence and risk factors for pulmonary embolism in the postpartum period. J Thromb Haemost. 2010;8(5):998–1003. 9. Creanga AA, Berg CJ, Syverson C, Seed K, Bruce FC, Callaghan WM. Pregnancy-related mortality in the United States, 2006-2010. Obstet Gynecol. 2015;125(1):5–12. 10. Kamel H, Navi BB, Sriram N, Hovsepian DA, Devereux RB, Elkind MS. Risk of a thrombotic event after the 6-week postpartum period. N Engl J Med. 2014;370(14):1307–15. 11. Blondon M, Harrington LB, Righini M, Boehlen F, Bounameaux H, Smith NL. Racial and ethnic differences in the risk of postpartum venous thromboembolism: a population-based, case-control study. J Thromb Haemost. 2014;12(12):2002–9. 12. Sultan AA, Tata LJ, West J, Fiaschi L, Fleming KM, Nelson-Piercy C, et al. Risk factors for first venous thromboembolism around pregnancy: a population-based cohort study from the United Kingdom. Blood. 2013;121(19):3953–61. 13. Simpson EL, Lawrenson RA, Nightingale AL, Farmer RD. Venous thromboembolism in pregnancy and the puerperium: incidence and additional risk factors from a London perinatal database. BJOG. 2001;108(1):56–60.

14. Abdul Sultan A, West J, Tata LJ, Fleming KM, Nelson-Piercy C, Grainge MJ. Risk of first venous thromboembolism in pregnant women in hospital: population based cohort study from England. BMJ. 2013;347:f6099. 15. American College of Obstetricians and Gynecologists Women’s Health Care Physicians. ACOG Practice Bulletin No. 138: inherited thrombophilias in pregnancy. Obstet Gynecol. 2013;122(3):706–17. 16. James A, Committee on Practice Bulletins—Obstetrics. Practice bulletin no. 123: thromboembolism in pregnancy. Obstet Gynecol. 2011;118(3):718–29. 17. Task Force on the Management of Cardiovascular Diseases During Pregnancy of the European Society of Cardiology. Expert consensus document on management of cardiovascular diseases during pregnancy. Eur Heart J. 2003;24(8):761–81. 18. Bates SM, Greer IA, Middeldorp S, Veenstra DL, Prabulos AM, Vandvik PO. VTE, thrombophilia, antithrombotic therapy, and pregnancy. Chest. 2012;141(2 Suppl):e691S–736S. 19. LeFevre ML, U.S. Preventive Services Task Force. Low-dose aspirin use for the prevention of morbidity and mortality from preeclampsia: U.S. Preventive Services Task Force recommendation statement. Ann Intern Med. 2014;161(11):819–26. 20. American College of Obstetricians and Gynecologists. Hypertension in pregnancy. Task force on hypertension in pregnancy. Washington, DC. 2013. http://www.acog.org/ResourcesAnd-Publications/Task-Force-and-Work-Group-Reports/ Hypertension-in-Pregnancy. 21. Gordon JL, Fabian TC, Lee MD, Dugdale M. Anticoagulant and antiplatelet medications encountered in emergency surgery patients: a review of reversal strategies. J Trauma Acute Care Surg. 2013; 75(3):475–86. 22. Aspirin prescribing information. BAYER INC., CONSUMER CARE Mississauga, Canada. http://www.bayer.ca/omr/online/ aspirinpmen16sep2014ctrl169398.pdf. 23. Reaume KT, Regal RE, Dorsch MP. Indications for dual antiplatelet therapy with aspirin and clopidogrel: evidence-based recommendations for use. Ann Pharmacother. 2008;42(4):550–7. 24. Hillis C, Crowther MA. Acute phase treatment of VTE: anticoagulation, including non-vitamin K antagonist oral anticoagulants. Thromb Haemost. 2015;113(6):1193–202. 25. Heparin sodium injection. Pfizer Canada Inc. 17300 Trans Canada Highway Kirkland, Quebec H9J 2M5. http://www.pfizer. ca/sites/g/files/g10017036/f/201505/Heparin-pm_ctl_175554_ Dec_16_2014_E.pdf. 26. Fox NS, Laughon SK, Bender SD, Saltzman DH, Rebarber A. Anti-factor Xa plasma levels in pregnant women receiving low molecular weight heparin thromboprophylaxis. Obstet Gynecol. 2008;112(4):884–9. 27. Coumadin (warfarin sodium) tablets prescribing information. Bristol-Myers Squibb, Princeton, NJ. http://packageinserts.bms. com/pi/pi_coumadin.pdf Accessed 17 June 2016. 28. Praxbind prescribing information. Boehringer Ingelheim Pharmaceuticals, Inc. Ridgefield, CT. http://docs.boehringeringelheim.com/Prescribing%20Information/PIs/Praxbind/Praxbind.pdf. 29. Hurst K, Lee R, Handa A. Quick reference guide to the new oral anticoagulants. J Vasc Surg. 2016;63(6):1653–7. 30. Moen V, Dahlgren N, Irestedt L. Severe neurological complications after central neuraxial blockades in Sweden 1990-1999. Anesthesiology. 2004;101:950–9. 31. Crawford JS. Some maternal complications of epidural analgesia for labour. Anaesthesia. 1985;40:1219–25. 32. Bateman BT, Mhyre JM, Ehrenfeld J, Kheterpal S, Abbey KR, Argalious M, et al. The risk and outcomes of epidural hematomas after perioperative and obstetric epidural catheterization: a report

8 Anticoagulation from the Multicenter Perioperative Outcomes Group Research Consortium. Anesth Analg. 2013;116(6):1380–5. 33. Vandermeulen EP, Van Aken H, Vermylen J. Anticoagulants and spinal-epidural anesthesia. Anesth Analg. 1994;79(6):1165–77. 34. Mammen EF, Comp PC, Gosselin R, Greenberg C, Hoots WK, Kessler CM, et al. PFA-100 system: a new method for assessment of platelet dysfunction. Semin Thromb Hemost. 1998;24(2):195–202. 35. Klein SM, Slaughter TF, Vail PT, Ginsberg B, El-Moalem HE, Alexander R, et al. Thromboelastography as a perioperative measure of anticoagulation resulting from low molecular weight hep-

33 arin: a comparison with anti-Xa concentrations. Anesth Analg. 2000;91(5):1091–5. 36. Suchman AL, Mushlin AI. How well does the activated partial thromboplastin time predict postoperative hemorrhage? JAMA. 1986;256:750–3. 37. Horlocker TT, Wedel DJ, Rowlingson JC, Enneking FK, Kopp SL, Benzon HT, et al. Regional anesthesia in the patient receiving antithrombotic or thrombolytic therapy: American Society of Regional Anesthesia and Pain Medicine Evidence-Based Guidelines (Third Edition). Reg Anesth Pain Med. 2010;35(1):64–101.

9

Antiphospholipid Syndrome Jonathan Paek and Shobana Chandrasekhar

Abbreviations

Signs and Symptoms

APS ASRA LMWH SLE

1. APS is commonly identified with a constellation of clinical findings which may allow a clinician to suspect the diagnosis [3]. 2. One or more episodes of unexplained arterial or venous thrombosis including deep vein thrombosis, pulmonary embolism, myocardial infarction, and cerebral infarction may have occurred. 3. There may also be a history of pregnancy-related issues including one or more unexplained fetal death >10 weeks gestation; one or more preterm deliveries due to severe preeclampsia, eclampsia, or placental insufficiency; and three or more consecutive unexplained spontaneous abortions. 4. Unexplained thrombocytopenia or abnormally coagulation studies may be present. 5. Physical findings such as livedo reticularis (mottled purple vascular pattern on the skin) may be notable. 6. Valvular heart lesions and false-positive syphilis test are other indicators of disease. 7. APS is commonly seen in patients diagnosed with systemic lupus erythematosus (SLE).

Antiphospholipid syndrome American Society of Regional Anesthesia Low molecular weight heparin Systemic lupus erythematosus

Presentation and Symptoms Description Antiphospholipid syndrome (APS) is characterized by the combination of venous or arterial thrombosis and the persistent elevation of antiphospholipid antibodies. These antibodies are specific for phospholipid-binding proteins, which, at the cellular level, are proteins used in signaling, trafficking, and metabolism [1]. The three main types that are commonly encountered by anesthesiologists are anticardiolipin antibodies, anti-beta-2-glycoprotein I antibody, and lupus anticoagulants. APS is associated with a high level of morbidity in the parturient and can result in consecutive spontaneous abortions, fetal demise, and preterm births. The pathogenesis of APS in pregnancy remains unclear but is thought to affect trophoblastic tissue and directly activate platelets, thromboxane A2, and endothelial cells, leading to thrombosis [2].

J. Paek, M.D. Department of Anesthesiology, Baylor Medical Center, Houston, TX, USA Baylor University School of Medicine, Houston, TX, USA e-mail: [email protected] S. Chandrasekhar, M.D. (*) Department of Anesthesiology, Baylor Medical Center, Houston, TX, USA e-mail: [email protected]

Incidence The incidence of APS is approximately 5 cases per 100,000, and the prevalence is approximately 40–50 cases per 100,000 people per year [4].

Interaction with Pregnancy Effect of Pregnancy on APS 1. It is currently unknown whether pregnancy has any direct effects on APS. 2. Given that pregnancy itself is a hypercoagulable state, it is likely that pregnancy can contribute to thrombosis in

© Springer International Publishing AG, part of Springer Nature 2018 S. K. W. Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_9

35

36

J. Paek and S. Chandrasekhar

patients with APS. Lima et al. showed that the incidence of thrombotic events in pregnant patients with known APS was as high as 12%, though there is not enough data to suggest that pregnancy directly increases the rate of thrombotic events in patients with APS [5].

2. While coagulation tests in the context of APS remain controversial, other tests such as a baseline platelet count, serum creatinine, urine protein to creatinine ratio, and liver function tests should be monitored throughout the delivery process.

Effect of APS on Pregnancy

Management

1. Unexplained fetal death is commonly seen in patients with APS. While the rate of fetal death prior to 10 weeks gestation remained similar between patients with and without APS, the rate of unexplained fetal death after 10 weeks gestation in patients with APS is three times higher [6]. Although many women have APS in conjunction with a diagnosis of SLE, the risk of fetal death seems to be related to the diagnosis of APS and independent of SLE [7]. 2. Recurrent pregnancy loss is also more commonly seen in APS, with women frequently having three or more consecutive spontaneous abortions. While no prospective studies have been conducted looking at the direct effect of APS on recurrent pregnancy loss, observational studies have noted that patients with recurrent spontaneous abortions were much more likely to have antiphospholipid antibody than compared to those with successful pregnancies (16% vs. 7%) [8]. 3. Preeclampsia is thought to be related to the presence of antiphospholipid antibody, though the data on the relationship is extremely limited. While the relationship of preeclampsia with anticardiolipin antibody, anti-beta-­2glycoprotein-I antibody, and lupus anticoagulant has been seen in several studies, no definitive link between preeclampsia and antiphospholipid antibody has been confirmed [9].

Medical and Obstetric Management

Testing 1. Testing for antiphospholipid antibody includes three separate tests for each antibody. Anticardiolipin and anti-beta-2-glycoprotein I antibodies are tested through ELISA, testing for the presence of each respective IgG or IgM antibody. Lupus anticoagulant testing is done through a functional assay with a mixing study using either the dilute Russell viper venom time or activated partial thromboplastin time. A prolonged test indicates the presence of lupus anticoagulant. Testing for antiphospholipid antibodies usually occurs when there is some clinical suspicion for the diagnosis of APS (discussed above). Confirmatory testing should occur 12 weeks after initial testing to confirm the presence of the antibody. Diagnosis of APS is through the revised Sapporo APS classification criteria and requires the presence of antiphospholipid antibodies and the presence of clinical criteria (vascular thrombosis, pregnancy morbidity).

1. The most important therapeutic goal in APS is prevention of thrombosis. Thromboprophylaxis differs for patients who have never had a thrombotic event versus those with a personal history of thrombosis. Low-dose aspirin is recommended as the primary prophylaxis for women without a history of a thrombotic event. Aspirin can be stopped after 36 weeks gestation and should ideally be stopped 7–10 days prior to delivery. In the non-gravid state, for secondary prophylaxis, patients use warfarin for a target INR of 2.0–3.0 [10]; however, during pregnancy low molecular weight heparin (LMWH) should be used as warfarin has the potential for serious complications to the fetus. 2. LMWH has been shown to be safe for the treatment of thromboembolism during pregnancy [11]. LMWH is a great option for prophylaxis throughout pregnancy, but it may be more appropriate to switch to unfractionated heparin during the final weeks of pregnancy because of the ease of reversibility of unfractionated heparin. Unfractionated heparin has been shown to have no differences in pregnancy outcomes compared to LMWH when both are combined with aspirin in the context of APS [12]. In addition, low-dose heparin (5000u BID) has been shown to have equal efficacy to high-dose heparin (10,000u BID) in preventing thromboses in APS patients [13]. Heparin is considered very safe during pregnancy as it does not cross the placenta and, in conjunction with aspirin, is the treatment of choice for thromboprophylaxis. 3. While heparin is the treatment of choice in APS, those patients who are not candidates for heparin are limited for choice in terms of anticoagulation. Recent studies have suggested that dipyridamole and hydroxychloroquine are potential options for anticoagulation during pregnancy, which can reduce the magnitude of platelet activation caused by APS [14]. Agents such as direct thrombin inhibitors and direct factor Xa inhibitors should be avoided due to the lack of data on their use during pregnancy.

Anesthetic Management 1. In parturients undergoing spontaneous vaginal delivery, labor epidural requires excellent coordination with the obstetric service after admission of patient to labor and

9  Antiphospholipid Syndrome

delivery. Aspirin therapy alone is not considered a contraindication to neuraxial anesthesia. The anesthesiologist should evaluate comorbidities such as secondary organ involvement and previous thrombotic episodes. Coexisting lupus should be evaluated if present. 2. The following guidelines are for the use of neuraxial anesthesia in the context of anticoagulation [15]. Please see the section on ASRA guidelines for further details. Twelve to 24 h should elapse since the last dose of LMWH, depending on the dose, prior to placement on neuraxial analgesia and anesthesia. After placement of an epidural catheter, LMWH therapy should be restarted no earlier than 12 h since the neuraxial block. Catheter removal should be done at least 12 h after the last dose of LMWH. After removal of catheter, LMWH can be re started after 4 h. 3. In patients receiving unfractionated heparin, neuraxial block can be placed 4 h after the last dose. Coagulation studies are advised. After removal of the catheter, heparin can be restarted immediately. Platelet values should be monitored for heparin-induced thrombocytopenia.

Postoperative Management 1. Catastrophic APS is a rare but extremely fatal complication during the postoperative period. It is defined by three characteristics: thromboses in three or more organs developing over the course of 1 week, microthrombi in at least one organ, and persistently present antiphospholipid antibody. Mortality from catastrophic APS is extremely high. Major triggers include infection and surgical stress. Care should be taken to address any surgical site infections, and anticoagulation should be continued into the postoperative period. Steroids have also been postulated to protect against catastrophic APS by decreasing cytokine release from necrotic tissue [16]. 2. Women with APS who have had a previous thrombotic event should continue lifelong warfarin therapy. The benefit of anticoagulation for women who have not had a previous thrombotic event is unclear. While no definitive practice guidelines exist, patients who received aspirin and heparin throughout the peripartum period continue this regiment for 6 weeks postpartum. 3. Despite thrombophylaxis with heparin, treatment failure is a tangible risk, and there are currently no effective second-­ line therapies. IVIG and therapeutic plasma exchange have not yielded success in prevention of pregnancy loss in treatment failure with heparin. Glucocorticoids have also been considered. Glucocorticoids in the peripartum period have risks such as preterm delivery, maternal hyperglycemia, intrauterine growth restriction, and infection. As discussed

37

above, hydroxychloroquine is a potential treatment that has been shown to reverse platelet activation, but its safety profile in humans must be further explored.

References 1. Hurley JH, Tsujishita Y, Pearson MA. Floundering about at cell membranes: a structural view of phospholipid signaling. Curr Opin Struct Biol. 2000;10:737–43. 2. Tong M, Viall CA, Chamley LW. Antiphospholipid antibodies and the placenta: a systematic review of their in vitro effects and modulation by treatment. Hum Reprod Update. 2015;21(1):97–118. 3. Miyakis S, Lockshin M, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4:295–306. 4. Gómez-Puerta JA, Cervera R. Diagnosis and classification of the antiphospholipid syndrome. J Autoimmun. 2014;48–49:20–5. 5. Lima F, Khamastha MA, Buchanan NM, et al. A study of sixty pregnancies in patients with the antiphospholipid syndrome. Clin Exp Rheumatol. 1996;14:131–6. 6. Loizou S, Byron MA, Englert HJ, et al. Association of quantitative anticardiolipin antibody levels with fetal loss and time of loss in systemic lupus erythematosus. Q J Med. 1988;68:525–31. 7. McNeil HP, Chesterman CN, Krilis SA. Immunology and clinical importance of antiphosphlipid antibodies. Adv Immunol. 1991;49:193–280. 8. Parke AL, Wilson D, Maier D. The prevalence of antiphospholipid antibodies in women with recurrent spontaneous abortion, women with successful pregnancies, and women who have never been pregnant. Arthritis Rheum. 1991;34:1231–5. 9. Prado AD, Piovesan DM, Staub HL, Horta BL. Association of anticardiolipin antibodies with preeclampsia: a systematic review and meta-analysis. Obstet Gynecol. 2010;116:1433–43. 10. Ruiz-Irastorza G, Crowther M, Branch W, Khamastha MA. Antiphospholipid syndrome. Lancet. 2010;367:1498–509. 11. Greer IA, Nelson-Piercy C. Low-molecular-weight heparins for thromboprophylaxis and treatment of venous thromboembolism in pregnancy: a systematic review of safety and efficacy. Blood. 2005;106:401–7. 12. Noble LS, Kutteh WH, Lashey N, et al. Antiphospholipid antibodies associated with recurrent pregnancy loss: prospective, multicenter, controlled pilot study comparing treatment with low-­ molecular-­ weight heparin versus unfractionated heparin. Fertil Steril. 2005;83:684–90. 13. Kutteh WH, Ermel LD. A clinical trial for the treatment of antiphospholipid-­antibody associated recurrent pregnancy loss with lower dose heparin and aspirin. Am J Reprod Immunol. 1996;35:402–7. 14. Erkan D, Yazici Y, Peterson MG, Sammaritano L, Lockshin MD. A cross-sectional study of clinical thrombotic risk factors and preventive treatments in antiphospholipid syndrome. Rheumatology. 2002;41:924–9. 15. Horlocker TT, Wedel DJ, Rowlingson JC, et al. Regional anesthesia in the patient receiving antithrombotic or thrombolytic therapy. American Society of Regional Anesthesia and Pain Medicine Evidence-Based Guidelines (Third edition). Reg Anesth Pain Med. 2010;35:64–101. 16. Cevera R, Bucciarelli S, Plasin MA, Gomez-Puerta JA, Plaza J, Pons-Estel G, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the “CAPS Registry”. J Autoimmun. 2009;32:240–5.

Antithrombin Deficiency

10

James P. R. Brown and Joanne Douglas

Background: Epidemiology, Pathophysiology

3 . Type 2 is a qualitative defect. 4. The homozygous genotype is usually fatal in utero.

Formally known as antithrombin III deficiency.

Incidence [3] Pathophysiology 1. The incidence is 1 in 500 to 1 in 5000. 1. Antithrombin (AT) binds and deactivates thrombin, factors IXa, Xa, XIa, and XIIa. 2. AT decreases the production and half-life of thrombin. 3. Acquired AT deficiency can occur from decreased synthesis (liver disease), consumption (disseminated intravascular coagulation), or urinary loss (nephrotic syndrome). AT deficiency may also be pregnancy-induced most commonly with multifetal gestations [1]. 4. Heparin potentiates the action of AT by more than 1000-fold.

Genetics [2] 1. Inheritance is in an autosomal dominant manner. There are many different mutations (>250), which result in a phenotypic range of expression: 40–70% of normal AT levels. 2. Type 1 is a quantitative deficiency.

Thrombosis Risk per Pregnancy [3, 4] 1. AT deficiency has the highest incidence of VTE out of all inherited thrombophilias with a lifetime thrombosis risk of 50%. 2. Without a personal history of VTE, the thrombosis risk is 3–7%. 3. With a personal history of VTE, the thrombosis risk is 40%.

Management Testing 1 . A functional activity assay is diagnostic if 2 days/ week; not daily

More than 2 times/ month

More than 80%

Daily

More than once/week

60–80%

Throughout the day

4 times/ week or mare

30%

Medications Required To Maintain Long-Term Control

Daily Medications

• Preferred treatment: - High-dose inhaled corticosteroid AND - Long-acting inhaled beta2-agonist AND, if needed - Corticosteroid tablets or syrub long term (2 mg/kg per day, generally not to exceed 60 mg per day). (Make repeat attempts to reduce systemic corticosteroid and maintain control with high-dose inhaled corticosteroid.*) • Alternative treatment: - High-dose inhaled corticosteroid* AND - Sustained release theophylline to serum concentration of 5–12 mcg/mL.

Daily Step 3 Moderate >1 night/week Persistent

≤60%–30%

Intermittent

Quick Relief

- Low-dose inhaled corticosteroid* and long-acting inhaled beta2-agonist OR

- Medium-dose inhaled corticosteroid* if needed (particularly in patients with recurring severe exacerbations): - Medium-dose inhaled corticosteroid* and long-acting inhaled beta2-agonist • Alternative treatment: - Low-dose inhaled corticosteroid* and either theophylline or leukotriene receptor antagonist. † If needed: - Medium-does inhaled corticosteroid* and either theophylline or leukotriene receptor antagonist.†

>2 days/week Step 2 ≥80% but 2 nights/month 20%–30% Step 1 Mild

• Preferred treatment: EITHER

≤2 days/week ≥80% ≤2 nights/month 2 times a week in intermittent asthma (daily, or increasing use in persistent asthma) may indicate the need ti initiate (increase) long-term-control therapy.

Step down Review treatment every 1–6 months; a gradual stepwise reduction in treatment may be possible. Step up If control is not maintained, consider step up. First, review patient medication technique, adherence, and environmental control.

Goals of Therapy: Asthma Control • • Minimal or no chronic symptoms day or night • Minimal or no exacerbations • • No limitations on activities; no school/work missed

Maintain (near) normal pulmonary function Minimal use of shortacting inhaled beta2agonist ‡ • Minimal or no adverse effects from medications

Notes • The stepwise approach is meant to assist, not replace, the clinical decisionmaking required to meet individual patient need.

• Classify severity: assign patient to most severe step in which any feature occurs (PEF is percent of personal best: FEV1 is percent predicted)

• Gain control as quickly as possible (consider a short course of systemic

corticosteroid). then step down to the least medication necessary to maintain control. • Minimize use of short-acting inhaled beta2-agonist‡ (e.g., use of approximately one canister a month even if not using it every day indicates inadequate control of asthma and the need to initiate or intensity long-term-control therapy) • Provide education on self-management and controlling environmental factors that make asthma worse (e.g., allergens, irritants). • Refer to an asthma specialist if there are difficulties controlling asthma or if Step 4 care is required. Referral may be considered if step 3 care is required.

* There are more data on using budesonide during pregnancy than using other inhaled corticosterolds

† There are minimal data on using leukotriene receptor antagonist in humans during pregnancy, althhough there are reassuring animal data submitted to FDA ‡ There are more data on using lbuterol during pregnancy than on using other short-acting inhaled beta2-agonists

Fig. 16.1  Pharmacologic treatment of asthma during pregnancy. From NAEPP expert panel report: managing asthma during pregnancy: recommendations for pharmacologic treatment—update 2004. Report can

be accessed at: http://www.nhlbi.nih.gov/health/prof/lung/asthma/astpreg.htm. Last accessed March 2016

66

S. K. W. Mankowitz Figure 5

Management of Asthma Exacerbations During Pregnancy and Location: Emergency Department and Hospital-Based Care* Initial Assessment

History, physical examination (auscultation, use of accessory muscles, heart rate, respiratory rate), PEF or FEV1, oxygen saturation, and other test as indicated Initiate fetal assessment (consider continuous electronic fetal monitoring and/or biophysical profile if pregnancy has reached fetal viability)

FEV1 or PEF >50% • Short-acting inhaled beta2-agonist by MDI or nebulizer, up to three does in first hour • Oxygen to achieve O2 saturation ≥95% • Oral systemic corticosteroid if no immediate response or if patient recently took oral systemic corticosteroid

FEV1 or PEF 95% • Oral systemic corticosteroid

Impending or Actual Respiratory Arrest • Intubation and mechanical ventilation with 100% O2 • Nebulized short-acting inhaled beta2agonist plus inhaled ipratropium bromide • Intravenous corticosteroid

Repeat Assessment Symptoms, physical examination, PEF, O2 saturation, other tests as needed Continue fetal assessment

Moderate Exacerbation FEV1 or PEF 50%–80% predicted/personal best Physical exam: moderate symptoms • Short-acting inhaled beta2-agonist every 60 minutes • Systemic corticosteroid • Oxygen to maintain O2 saturation >95% • Continue treatment 1–3 hours, proved there is improvement

Good Response • FEV1 or PEF ≥70% • Response sustained 60 minutes after last treatment • No distress • Physical exam: normal • Reassuring fetal status

Admit to Hospital Intensive Care (see box below)

Serve Exacerbation FEV1 or PEF 10 mm). If significant growth occurs, elevated intracranial pressure (ICP) may result. 4. If the tumor expands rapidly, it may compress its own blood supply, resulting in ischemia, hemorrhage, and pituitary apoplexy. This is a neurosurgical emergency. Pituitary apoplexy during pregnancy primarily affects women with no prior pituitary diagnosis [7].

C. Traill and S. H. Halpern

3. In pregnant patients with a known pituitary adenoma who present with persistent headaches or visual field disturbance, an MRI may be required to assess tumor size. Surgical resection via a transsphenoidal approach may be necessary [8]. 4. In patients with a non-secreting tumor who present with headaches or mild visual field changes only, a trial of corticosteroids and bromocriptine may be commenced in an attempt to improve symptoms and delay surgical removal until after delivery [1]. 5. In patients who present with pituitary apoplexy, conservative treatment with corticosteroids and bromocriptine may be undertaken in mild cases, but in the case of progressive symptoms, surgical resection is likely necessary [1].

Anesthetic Management Medication 1. Postpartum hypertension, seizures, and stroke have been reported in women taking bromocriptine in the postpartum period for lactation suppression [9].

Neuraxial Analgesia Effects of Pituitary Adenoma on Pregnancy 1. The effects of pituitary adenomas on pregnancy depend upon the specific hormones secreted by the adenoma.

Medical Management Testing and Diagnosis 1. Secretory pituitary adenomas may be diagnosed on the basis of a persistently elevated serum hormone level in conjunction with MRI of the brain. 2. Nonsecretory adenomas and pituitary apoplexy may be diagnosed on neuroimaging alone. 3. In some cases, a transsphenoidal biopsy may be necessary to confirm diagnosis, although this is generally avoided during pregnancy.

1. Neuraxial analgesia is safe for patients with pituitary adenomas, provided there are no signs of raised intracranial pressure. 2. In patients with raised ICP, the benefit of neuraxial anesthesia in limiting ICP increases during contractions, and pushing must be weighed against the potential for brain herniation with an inadvertent dural puncture. Ideally this would be a collaborative decision between anesthetic, obstetric, and neurologic teams and would be made prior to the patient presenting in labor. 3. While epidural and spinal anesthesia using small-gauge spinal needles have been used successfully in pregnant patients with raised ICP, there are two reports of patients with supratentorial mass lesions experiencing fatal brain herniation after epidural placement for labor [10, 11].

Anesthesia for Cesarean Delivery Medical Management 1. Initially, conservative management is recommended when a pregnant patient presents with a pituitary adenoma [1]. 2. Specific management options depend upon the type of adenoma present and the hormone involved.

1. Both regional and general anesthetic techniques have been used safely for Cesarean delivery in patients with pituitary adenomas without raised ICP. 2. In patients with raised ICP, the potential risk of brain herniation due to dural puncture with a neuraxial technique must be weighed against the risk of neurological deterioration that may occur with general anesthesia. Ideally, a

124  Pituitary Adenoma

multidisciplinary discussion should take place with ­anesthesia, obstetric, neurologic, and neonatal teams, and a decision made for each individual case [12]. 3. If general anesthesia is performed, the sympathetic response to intubation should be blocked by combining an induction agent with an opioid (such as fentanyl 2–5 μg/kg or remifentanil 1 μg/kg over 1 min) or labetalol in 5 mg boluses. If opioids are used, the neonatal team must be informed. A non-depolarizing muscle relaxant such as rocuronium 1.2 mg/kg may be preferable to suxamethonium which produces a transient increase in ICP due to thoracic and abdominal muscle contraction. Care must also be taken during extubation to prevent sympathetic stimulation and coughing. This can be achieved with the use of a short-acting opioid infusion such as remifentanil or with antihypertensive agents such as labetalol. 4. To balance maternal and fetal cerebral perfusion during general anesthesia, maternal blood pressure should be monitored through the use of an arterial line and kept close to baseline. Ventilation should be altered to produce a maternal PCO2 of 25–30 mmHg [12]. The patient should be positioned head up 15° in addition to a left lateral tilt.

Postoperative Management 1. In asymptomatic patients, standard postoperative care is adequate. 2. In patients with raised ICP, regular neurological monitoring should be performed in the first 24 h post-delivery to assess for any neurological deterioration.

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References 1. Iuliano S, Laws ER Jr. Management of pituitary tumors in pregnancy. Semin Neurol. 2011;31(4):423–8. 2. Ezzat S, Asa SL, Couldwell WT, Barr CE, Dodge WE, Vance ML, McCutcheon IE. The prevalence of pituitary adenomas. Cancer. 2004;101(3):613–9. 3. Karaca Z, Kelestimur F. Pregnancy and other pituitary disorders (including GH deficiency). Best Pract Res Clin Endocrinol Metab. 2011;25(6):897–910. 4. Scheithauer BW, Sano T, Kovacs KT, Young WF, Ryan N, Randall RV. The pituitary gland in pregnancy: a clinicopathologic and immunohistochemical study of 69 cases. Mayo Clin Proc. 1990;65(4):461–74. 5. Dinc H, Esen F, Demirci A, Sari A, Gumele HR. Pituitary dimensions and volume measurements in in pregnancy and postpartum MR assessment. Acta Radiol. 1998;39(1):64–9. 6. Karaca Z, Tanriverdi F, Unluhizarci K, Kelestimur F. Pregnancy and pituitary disorders. Eur J Endocrinol. 2010;162(3): 453–75. 7. Laws ER. Pituitary tumor apoplexy: a review. J Intensive Care Med. 2008;23(2):146–7. 8. Kupersmith MJ, Rosenberg C, Kleinberg D. Visual loss in pregnant women with pituitary adenomas. Ann Intern Med. 1994;121(7):473–7. 9. Katz M, Kroll D, Pak I, Osimoni A, Hirsch M. Peurperal hypertension, stroke, and seizures after suppression of lactation with bromocriptine. Obstet Gynecol. 1985;66(6):822–4. 10. Su TM, Lan CM, Yang LC, Lee TC, Wang KW, Hung KS. Brain tumor presenting with fatal herniation following delivery under epidural anesthesia. Anesthesiology. 2002;96(2):508–9. 11. Goroszeniuk T, Howard RS, Wright JT. The management of labour using continuous lumbar epidural analgesia in a patient with a malignant cerebral tumour. Anaesthesia. 1986;41(11): 1128–9. 12. Leffert LR, Schwamm LH. Neuraxial anesthesia in parturients with intracranial pathology: a comprehensive review and reassessment of risk. Anesthesiology. 2013;119(3):703–18.

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Polycythemia Vera Andrea Girnius and Lesley Gilbertson

Background 1. Definition: Polycythemia vera (PV) is a chronic myeloproliferative neoplasm characterized by increased red blood cell mass. It is almost universally associated with a Janus-kinase 2 (JAK2) gene mutation. This gene is involved in intracellular cytokine and growth factor signaling in hematopoietic cells. Diagnosis requires the presence of both major criteria and one minor criterion OR the first major criterion and two minor criteria [1]. (a) Major criteria • Hemoglobin >18.5 g/dL in men or >16.5 g/dL in women, or other evidence of increased red cell volume • Presence of JAK2V617F or other functionally similar mutation (b) Minor criteria • Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) with prominent erythroid, granulocytic, and megakaryocytic proliferation • Serum erythropoietin level below the reference range for normal • Endogenous erythroid colony formation in vitro 2. Incidence: The overall reported incidence of PV is 2/100,000 people [2]. Fewer than 50 cases have been reported during pregnancy [3]. Median survival is 14.1 years, with mortality mainly due to thrombotic A. Girnius, M.D. (*) Department of Anesthesiology, University of Cincinnati Medical Center, Cincinnati, OH, USA e-mail: [email protected] L. Gilbertson, M.D. Division Director Obstetric Anesthesia, University of Cincinnati Medical Center and West Chester Hospital, Cincinnati, OH, USA e-mail: [email protected]

complications or progression to leukemia or myelofibrosis [4]. 3. Symptoms: PV is most commonly discovered incidentally when a routine complete blood count shows an elevated hematocrit. Common presenting symptoms include headache, dizziness, visual disturbances, pruritus (especially after exposure to warm water), early satiety, erythromelalgia, claudication, or angina. Patients may also present with arterial or venous thrombosis (including stroke or myocardial infarction) or hemorrhage [4]. Signs on physical examination may include splenomegaly, hepatomegaly, plethora, and hypertension. 4. Rarely, PV can be associated with acquired von Willebrand disease (vWD). This is thought to be due to adsorption of von Willebrand factor (vWF) to abnormal blood cells [5].

Interaction with Pregnancy Effects of Pregnancy on Polycythemia Vera 1. Hematologic changes that occur during pregnancy include decreased relative red cell mass; increases in circulating levels of fibrinogen, vWF, and factors VII, VIII, and X; decrease in protein S; and subsequent acquired activated protein C resistance. The overall hypercoagulable state of pregnancy can interact negatively with the hypercoagulable state of PV. 2. The increase in vWF seen in pregnancy may mitigate the effects of acquired vWD, although acquired vWD has never been reported in a pregnant patient with PV. 3. Untreated PV patients have increased plasma volume and therefore increased cardiac output (CO). The further increase in CO seen during pregnancy may increase the risk of high output heart failure.

© Springer International Publishing AG, part of Springer Nature 2018 S. K. W. Mankowitz (ed.), Consults in Obstetric Anesthesiology, https://doi.org/10.1007/978-3-319-59680-8_125

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Effects of Polycythemia Vera on Pregnancy [6] 1. Untreated PV is associated with a live birth rate of 55%. This may be improved with treatment, although the paucity of literature precludes definitive conclusions [6]. 2. First trimester pregnancy loss appears to be more common [7]. 3. Reported fetal complications include intrauterine growth restriction (IUGR) and premature birth. 4. Maternal complications include thrombosis, pre-­eclampsia, hemorrhage, and death.

Management Medical Management 1. The treatment of PV is centered around reduction of red cell volume to correct the increased blood viscosity and red blood cell aggregation caused by an elevated hematocrit. This can be accomplished with serial phlebotomy in low-risk patients and phlebotomy with cytotoxic medications in high-risk patients. Hydroxyurea is the most common cytotoxic medication, although interferon-α, busulfan, and ruxolitinib can be used in refractory patients [4]. Maintenance of the hematocrit